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Artículos originales (todos) *** Original articles (all)

Neuroendocrine tumors.

December 2012 - January 2013

 

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[1]

TÍTULO / TITLE:  - Screening for insulinoma antigen 2 and zinc transporter 8 autoantibodies: a cost-effective and age-independent strategy to identify rapid progressors to clinical onset among relatives of type 1 diabetic patients.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Clin Exp Immunol. 2013 Jan;171(1):82-90. doi: 10.1111/j.1365-2249.2012.04675.x.

            ●● Enlace al texto completo (gratuito o de pago) 1111/j.1365-2249.2012.04675.x

AUTORES / AUTHORS:  - Gorus FK; Balti EV; Vermeulen I; Demeester S; Van Dalem A; Costa O; Dorchy H; Tenoutasse S; Mouraux T; De Block C; Gillard P; Decochez K; Wenzlau JM; Hutton JC; Pipeleers DG; Weets I

INSTITUCIÓN / INSTITUTION:  - Diabetes Research Center, Brussels Free University, Brussels, Belgium.

RESUMEN / SUMMARY:  - In first-degree relatives of type 1 diabetic patients, we investigated whether diabetes risk assessment solely based on insulinoma antigen 2 (IA-2) and zinc transporter 8 (ZnT8) antibody status (IA-2A, respectively, ZnT8A) is as effective as screening for three or four autoantibodies [antibodies against insulin (IAA),  glutamate decarboxylase 65 kDa (GAD) glutamate decarboxylase autoantibodies (GADA) and IA-2A with or without ZnT8A] in identifying children, adolescents and  adults who progress rapidly to diabetes (within 5 years). Antibodies were determined by radiobinding assays during follow-up of 6444 siblings and offspring aged 0-39 years at inclusion and recruited consecutively by the Belgian Diabetes  Registry. We identified 394 persistently IAA(+) , GADA(+) , IA-2A(+) and/or ZnT8A(+) relatives (6.1%). After a median follow-up time of 52 months, 132 relatives developed type 1 diabetes. In each age category tested (0-9, 10-19 and  20-39 years) progression to diabetes was significantly quicker in the presence of IA-2A and/or ZnT8A than in their joint absence (P < 0.001). Progression rate was  age-independent in IA-2A(+) and/or ZnT8A(+) relatives but decreased with age if only GADA and/or IAA were present (P = 0.008). In the age group mainly considered for immune interventions until now (10-39 years), screening for IA-2A and ZnT8A alone identified 78% of the rapid progressors (versus 75% if positive for >/= 2 antibodies among IAA, GADA, IA-2A and ZnT8A or versus 62% without testing for ZnT8A). Screening for IA-2A and ZnT8A alone allows identification of the majority of rapidly progressing prediabetic siblings and offspring regardless of age and is more cost-effective to select participants for intervention trials than conventional screening.

 

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[2]

TÍTULO / TITLE:  - MEK inhibition exhibits efficacy in human and mouse neurofibromatosis tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Clin Invest. 2013 Jan 2;123(1):340-7. doi: 10.1172/JCI60578. Epub 2012 Dec 10.

            ●● Enlace al texto completo (gratuito o de pago) 1172/JCI60578

AUTORES / AUTHORS:  - Jessen WJ; Miller SJ; Jousma E; Wu J; Rizvi TA; Brundage ME; Eaves D; Widemann B; Kim MO; Dombi E; Sabo J; Hardiman Dudley A; Niwa-Kawakita M; Page GP; Giovannini M; Aronow BJ; Cripe TP; Ratner N

RESUMEN / SUMMARY:  - Neurofibromatosis type 1 (NF1) patients develop benign neurofibromas and malignant peripheral nerve sheath tumors (MPNST). These incurable peripheral nerve tumors result from loss of NF1 tumor suppressor gene function, causing hyperactive Ras signaling. Activated Ras controls numerous downstream effectors,  but specific pathways mediating the effects of hyperactive Ras in NF1 tumors are  unknown. We performed cross-species transcriptome analyses of mouse and human neurofibromas and MPNSTs and identified global negative feedback of genes that regulate Ras/Raf/MEK/ERK signaling in both species. Nonetheless, ERK activation was sustained in mouse and human neurofibromas and MPNST. We used a highly selective pharmacological inhibitor of MEK, PD0325901, to test whether sustained  Ras/Raf/MEK/ERK signaling contributes to neurofibroma growth in a neurofibromatosis mouse model (Nf1fl/fl;Dhh-Cre) or in NF1 patient MPNST cell xenografts. PD0325901 treatment reduced aberrantly proliferating cells in neurofibroma and MPNST, prolonged survival of mice implanted with human MPNST cells, and shrank neurofibromas in more than 80% of mice tested. Our data demonstrate that deregulated Ras/ERK signaling is critical for the growth of NF1  peripheral nerve tumors and provide a strong rationale for testing MEK inhibitors in NF1 clinical trials.

 

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[3]

TÍTULO / TITLE:  - Circulating tumor cells as prognostic markers in neuroendocrine tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Clin Oncol. 2013 Jan 20;31(3):365-72. doi: 10.1200/JCO.2012.44.2905. Epub 2012  Dec 17.

            ●● Enlace al texto completo (gratuito o de pago) 1200/JCO.2012.44.2905

AUTORES / AUTHORS:  - Khan MS; Kirkwood A; Tsigani T; Garcia-Hernandez J; Hartley JA; Caplin ME; Meyer T

INSTITUCIÓN / INSTITUTION:  - UCL Cancer Institute, University College London, 72 Huntley St, London WC1E 6BT United Kingdom; t.meyer@ucl.ac.uk.

RESUMEN / SUMMARY:  - PURPOSE To determine the prognostic significance of circulating tumor cells (CTCs) in patients with neuroendocrine cancer. PATIENTS AND METHODS In this single-center prospective study, 176 patients with measurable metastatic neuroendocrine tumors (NETs) were recruited. CTCs were measured using a semiautomated technique based on immunomagnetic separation of epithelial cell adhesion molecule-expressing cells. Results Overall, 49% patients had >/= one CTC, 42% had >/= two CTCs, and 30% had >/= five CTCs in 7.5 mL blood. Presence of CTCs was associated with increased burden, increased tumor grade, and elevated serum chromogranin A (CgA). Using a 90-patient training set and 85-patient validation set, we defined a cutoff of < one or >/= one as the optimal prognostic threshold with respect to progression-free survival (PFS). Applying this threshold, the presence of >/= one CTC was associated with worse PFS and overall  survival (OS; hazard ratios [HRs], 6.6 and 8.0, respectively; both P < .001). In  multivariate analysis, CTCs remained significant when other prognostic markers, grade, tumor burden, and CgA were included. Within grades, presence of CTCs was able to define a poor prognostic subgroup. For grade 1, HRs were 5.0 for PFS (P = .017) and 7.2 for OS (P = .023); for grade 2, HRs were 3.5 for PFS (P = .018) and 5.2 for OS (P = .036). CONCLUSION CTCs are a promising prognostic marker for patients with NETs and should be assessed in the context of clinical trials with  defined tumor subtypes and therapy.

 

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[4]

TÍTULO / TITLE:  - Differential gene expression of medullary thyroid carcinoma reveals specific markers associated with genetic conditions.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Am J Pathol. 2013 Feb;182(2):350-62. doi: 10.1016/j.ajpath.2012.10.025. Epub 2012 Nov 28.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.ajpath.2012.10.025

AUTORES / AUTHORS:  - Maliszewska A; Leandro-Garcia LJ; Castelblanco E; Macia A; de Cubas A; Gomez-Lopez G; Inglada-Perez L; Alvarez-Escola C; De la Vega L; Leton R; Gomez-Grana A; Landa I; Cascon A; Rodriguez-Antona C; Borrego S; Zane M; Schiavi F; Merante-Boschin I; Pelizzo MR; Pisano DG; Opocher G; Matias-Guiu X; Encinas M; Robledo M

INSTITUCIÓN / INSTITUTION:  - Hereditary Endocrine Cancer Group, Human Cancer Genetics Program, Spanish National Cancer Research Center (CNIO), Madrid, España.

RESUMEN / SUMMARY:  - Medullary thyroid carcinoma accounts for 2% to 5% of thyroid malignancies, of which 75% are sporadic and the remaining 25% are hereditary and related to multiple endocrine neoplasia type 2 syndrome. Despite a genotype-phenotype correlation with specific germline RET mutations, knowledge of pathways specifically associated with each mutation and with non-RET-mutated sporadic MTC  remains lacking. Gene expression patterns have provided a tool for identifying molecular events related to specific tumor types and to different clinical features that could help identify novel therapeutic targets. Using transcriptional profiling of 49 frozen MTC specimens classified as RET mutation,  we identified PROM1, LOXL2, GFRA1, and DKK4 as related to RET(M918T) and GAL as related to RET(634) mutation. An independent series of 19 frozen and 23 formalin-fixed, paraffin-embedded (FFPE) MTCs was used for validation by RT-qPCR. Two tissue microarrays containing 69 MTCs were available for IHC assays. According to pathway enrichment analysis and gene ontology biological processes,  genes associated with the MTC(M918T) group were involved mainly in proliferative, cell adhesion, and general malignant metastatic effects and with Wnt, Notch, NFkappaB, JAK/Stat, and MAPK signaling pathways. Assays based on silencing of PROM1 by siRNAs performed in the MZ-CRC-1 cell line, harboring RET(M918T), caused an increase in apoptotic nuclei, suggesting that PROM1 is necessary for survival  of these cells. This is the first report of PROM1 overexpression among primary tumors.

 

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[5]

TÍTULO / TITLE:  - Combined Inhibition of mTORC1 and mTORC2 Signaling Pathways Is a Promising Therapeutic Option in Inhibiting Pheochromocytoma Tumor Growth: In Vitro and In Vivo Studies in Female Athymic Nude Mice.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endocrinology. 2013 Feb;154(2):646-655. Epub 2013 Jan 10.

            ●● Enlace al texto completo (gratuito o de pago) 1210/en.2012-1854

AUTORES / AUTHORS:  - Giubellino A; Bullova P; Nolting S; Turkova H; Powers JF; Liu Q; Guichard S; Tischler AS; Grossman AB; Pacak K

INSTITUCIÓN / INSTITUTION:  - MD, Reproductive and Adult Endocrinology Program, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10-CRC, 1E-3140, 10 Center Drive, Bethesda, Maryland, 20892-1109. giubella@mail.nih.gov; or Karel Pacak, MD, PhD, DSc, Senior Investigator, Chief,  Section on Medical Neuroendocrinology, Professor of Medicine, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, National Institutes of Health, Bethesda, Maryland, 20892-1109. E-mail: karel@mail.nih.gov.

RESUMEN / SUMMARY:  - Several lines of evidence, including the recent discovery of novel susceptibility genes, point out an important role for the mammalian target of rapamycin (mTOR) signaling pathway in the development of pheochromocytoma. Analyzing a set of pheochromocytomas from patients with different genetic backgrounds, we observed and confirmed a significant overexpression of key mTOR complex (mTORC) signaling  mediators. Using selective ATP-competitive inhibitors targeting both mTORC1 and mTORC2, we significantly arrested the in vitro cell proliferation and blocked migration of pheochromocytoma cells as a result of the pharmacological suppression of the Akt/mTOR signaling pathway. Moreover, AZD8055, a selective ATP-competitive dual mTORC1/2 small molecular inhibitor, significantly reduced the tumor burden in a model of metastatic pheochromocytoma using female athymic nude mice. This study suggests that targeting both mTORC1 and mTORC2 is a potentially rewarding strategy and supports the application of selective inhibitors in combinatorial drug regimens for metastatic pheochromocytoma.

 

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[6]

TÍTULO / TITLE:  - Bevacizumab Plus Temozolomide: A Novel Treatment Option for Advanced Neuroendocrine Tumors?

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Clin Oncol. 2013 Jan 28.

            ●● Enlace al texto completo (gratuito o de pago) 1200/JCO.2012.45.7523

AUTORES / AUTHORS:  - Koumarianou A; Kaltsas G

INSTITUCIÓN / INSTITUTION:  - Attikon University General Hospital, Athens, Greece.

 

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[7]

TÍTULO / TITLE:  - Livedo Reticularis in a Patient With Pheochromocytoma Resolving After Adrenalectomy.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Clin Endocrinol Metab. 2012 Dec 28.

            ●● Enlace al texto completo (gratuito o de pago) 1210/jc.2012-2842

AUTORES / AUTHORS:  - Buckley SA; Lessing JN; Mark NM

INSTITUCIÓN / INSTITUTION:  - University of Washington, Department of Internal Medicine, Seattle, Washington 98195.

RESUMEN / SUMMARY:  - Abstract Not Available.

 

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[8]

TÍTULO / TITLE:  - Pheochromocytoma and gastrointestinal stromal tumors in patients with neurofibromatosis type I.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Am J Med. 2013 Feb;126(2):174-80. doi: 10.1016/j.amjmed.2012.07.022.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.amjmed.2012.07.022

AUTORES / AUTHORS:  - Vlenterie M; Flucke U; Hofbauer LC; Timmers HJ; Gastmeier J; Aust DE; van der Graaf WT; Wesseling P; Eisenhofer G; Lenders JW

INSTITUCIÓN / INSTITUTION:  - Department of Medical Oncology, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.

RESUMEN / SUMMARY:  - BACKGROUND: Neurofibromatosis I may rarely predispose to pheochromocytoma and gastrointestinal stromal tumors. METHODS: A 59-year-old woman with neurofibromatosis I presented with pheochromocytoma of the left adrenal gland. During surgery, 3 gastrointestinal stromal tumors adjacent to the stomach and small intestine were removed. Despite appropriate thrombosis prophylaxis, the patient died of a pulmonary embolus 2 days postoperatively. The second patient, a 55-year-old man with neurofibromatosis I and bilateral pheochromocytomas, had several small gastrointestinal stromal tumors adjacent to the jejunum during surgery. A review of the literature was conducted to identify patients with neurofibromatosis I with concurrence of pheochromocytoma and gastrointestinal stromal tumors and to define the specific clinical features of these patients. RESULTS: In addition to our 2 patients, 12 other cases of neurofibromatosis I with concomitant occurrence of pheochromocytomas and gastrointestinal stromal tumors have been reported. Pheochromocytomas had adrenal locations in all patients. Two of the 14 patients had a mixed pheochromocytoma/ganglioneuroma. In  4 of the 14 patients, gastrointestinal stromal tumors were located along the stomach. The gastrointestinal stromal tumors in our 2 patients showed no somatic  mutations in KIT and PDGFRA genes. A pulmonary embolism was diagnosed in 4 patients. CONCLUSIONS: The simultaneous occurrence of pheochromocytoma and gastrointestinal stromal tumor should be considered in all patients with neurofibromatosis I presenting with an abdominal mass with symptoms suggestive of pheochromocytoma. Therefore, a pheochromocytoma should be excluded before a patient with neurofibromatosis I undergoes surgery for a gastrointestinal stromal tumor because an undiagnosed pheochromocytoma carries a high risk of life-threatening cardiovascular complications during surgery. Finally, this combination may be associated with an increased risk for thromboembolic events, but more studies are necessary to confirm this.

 

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[9]

TÍTULO / TITLE:  - Neuroendocrine carcinoma of the uterine cervix: The role of multimodality therapy in early-stage disease.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Gynecol Oncol. 2013 Jan 25. pii: S0090-8258(13)00024-3. doi: 10.1016/j.ygyno.2013.01.014.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.ygyno.2013.01.014

AUTORES / AUTHORS:  - McCann GA; Boutsicaris CE; Preston MM; Backes FJ; Eisenhauer EL; Fowler JM; Cohn DE; Copeland LJ; Salani R; O’Malley DM

INSTITUCIÓN / INSTITUTION:  - Department of Obstetrics and Gynecology, Division of Gynecologic Oncology.

RESUMEN / SUMMARY:  - BACKGROUND: Neuroendocrine carcinomas (NEC) of the cervix comprise only two percent of all cervical cancers. Prospective data is limited and treatment guidelines rely on retrospective reviews and literature from lung NEC. The objective of this study was to report our experience in the management of this rare disease. METHODS: This was an IRB-approved retrospective review. Study criteria included patients with cervical NEC diagnosed between 1990 and 2012. Demographic, treatment and survival data was collected. Progression free survival (PFS) and overall survival (OS) were assessed. RESULTS: Twenty-six patients met inclusion criteria. Advanced-stage disease (II-IV) was diagnosed in 58 percent (n=15) of patients. Of the eleven patients with stage I disease, five were treated with platinum-based neoadjuvant chemotherapy (NACT), six with initial radical surgery, and seven received adjuvant therapy including chemotherapy and/or radiation. Nine patients (82%) are currently without evidence of disease (NED). Patients with stage I disease had significantly improved PFS and OS compared to stages II-IV with a median OS that was not reached and 12.1 months, respectively (p=0.0013). The majority of stage I patients with lymph node metastasis and large tumors achieved durable remission with triple-modality therapy including NACT and surgery followed by adjuvant therapy. CONCLUSIONS: Cervical NEC is an aggressive disease associated with a high mortality rate. Patients with advanced-stage disease have a poor prognosis regardless of therapy. However, multimodality with consideration of triple-modality therapy in early-stage disease has the potential for complete response and long-term survival, supporting the goal of curative intent in these patients.

 

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[10]

TÍTULO / TITLE:  - Comparison of 68Ga-DOTANOC and 68Ga-DOTATATE PET/CT Within Patients with Gastroenteropancreatic Neuroendocrine Tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Nucl Med. 2013 Jan 7.

            ●● Enlace al texto completo (gratuito o de pago) 2967/jnumed.112.111724

AUTORES / AUTHORS:  - Wild D; Bomanji JB; Benkert P; Maecke H; Ell PJ; Reubi JC; Caplin ME

INSTITUCIÓN / INSTITUTION:  - Institute of Nuclear Medicine, University College Hospital, London, United Kingdom.

RESUMEN / SUMMARY:  - Somatostatin receptor PET tracers such as [(68)Ga-DOTA,1-Nal(3)]-octreotide ((68)Ga-DOTANOC) and [(68)Ga-DOTA,Tyr(3)]-octreotate ((68)Ga-DOTATATE) have shown promising results in patients with neuroendocrine tumors, with a higher lesion detection rate than is achieved with (18)F-fluorodihydroxyphenyl-l-alanine PET, somatostatin receptor SPECT, CT, or MR imaging. (68)Ga-DOTANOC has high affinity  for somatostatin receptor subtypes 2, 3, and 5 (sst(2,3,5)). It has a wider receptor binding profile than (68)Ga-DOTATATE, which is sst(2)-selective. The wider receptor binding profile might be advantageous for imaging because neuroendocrine tumors express different subtypes of somatostatin receptors. The goal of this study was to prospectively compare (68)Ga-DOTANOC and (68)Ga-DOTATATE PET/CT in the same patients with gastroenteropancreatic neuroendocrine tumors (GEP-NETs) and to evaluate the clinical impact of (68)Ga-DOTANOC PET/CT. METHODS: Eighteen patients with biopsy-proven GEP-NETs were evaluated with (68)Ga-DOTANOC and (68)Ga-DOTATATE using a randomized crossover design. Labeling of DOTANOC and DOTATATE with (68)Ga was standardized using a fully automated synthesis device. PET/CT findings were compared with 3-phase CT scans and in some patients with MR imaging, (18)F-FDG PET/CT, and histology. Uptake in organs and tumor lesions was quantified and compared by calculation of maximum standardized uptake values (SUVmax) using volume computer-assisted reading. RESULTS: Histology revealed low-grade GEP-NETs (G1) in 4 patients, intermediate grade (G2) in 7, and high grade (G3) in 7. (68)Ga-DOTANOC and (68)Ga-DOTATATE were false-negative in only 1 of 18 patients.  In total, 248 lesions were confirmed by cross-sectional and PET imaging. The lesion-based sensitivity of (68)Ga-DOTANOC PET was 93.5%, compared with 85.5% for (68)Ga-DOTATATE PET (P = 0.005). The better performance of (68)Ga-DOTANOC PET is  attributed mainly to the significantly higher detection rate of liver metastases  rather than tumor differentiation grade. Multivariate analysis revealed significantly higher SUVmax in G1 tumors than in G3 tumors (P = 0.009). This finding was less pronounced with (68)Ga-DOTANOC (P > 0.001). Altogether, (68)Ga-DOTANOC changed treatment in 3 of 18 patients (17%). CONCLUSION: The sst(2,3,5)-specific radiotracer (68)Ga-DOTANOC detected significantly more lesions than the sst(2)-specific radiotracer (68)Ga-DOTATATE in our patients with GEP-NETs. The clinical relevance of this finding has to be proven in larger studies.

 

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[11]

TÍTULO / TITLE:  - Validation of the EORTC QLQ-GINET21 questionnaire for assessing quality of life of patients with gastrointestinal neuroendocrine tumours.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Br J Cancer. 2013 Jan 15. doi: 10.1038/bjc.2012.560.

            ●● Enlace al texto completo (gratuito o de pago) 1038/bjc.2012.560

AUTORES / AUTHORS:  - Yadegarfar G; Friend L; Jones L; Plum LM; Ardill J; Taal B; Larsson G; Jeziorski K; Kwekkeboom D; Ramage JK

INSTITUCIÓN / INSTITUTION:  - 1] Department of Primary Care and Public Health, Imperial College London, London, UK [2] Department of Biostatistics, Kermanshah University of Medical Sciences, Kermanshah, Iran.

RESUMEN / SUMMARY:  - Background:Quality of life is an important end point in clinical trials, yet there are few quality of life questionnaires for neuroendocrine tumours.Methods:This international multicentre validation study assesses the QLQ-GINET21 Quality of Life Questionnaire in 253 patients with gastrointestinal neuroendocrine tumours. All patients were requested to complete two quality of life questionnaires - the EORTC Core Quality of Life questionnaire (QLQ-C30) and  the QLQ-GINET21 - at baseline, and at 3 and 6 months post-baseline; the psychometric properties of the questionnaire were then analysed.Results:Analysis  of QLQ-GINET21 scales confirmed appropriate aggregation of the items, except for  treatment-related symptoms, where weight gain showed low correlation with other questions in the scale; weight gain was therefore analysed as a single item. Internal consistency of scales using Cronbach’s alpha coefficient was >0.7 for all parts of the QLQ-GINET21 at 6 months. Intraclass correlation was >0.85 for all scales. Discriminant validity was confirmed, with values <0.70 for all scales compared with each other.Scores changed in accordance with alterations in performance status and in response to expected clinical changes after therapies.  Mean scores were similar for pancreatic and other tumours.Conclusion:The QLQ-GINET21 is a valid and responsive tool for assessing quality of life in the gut, pancreas and liver neuroendocrine tumours.British Journal of Cancer advance  online publication, 15 January 2013; doi:10.1038/bjc.2012.560 www.bjcancer.com.

 

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[12]

TÍTULO / TITLE:  - Bevacizumab and Subtype-Adapted Chemotherapy Backbone in Neuroendocrine Tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Clin Oncol. 2013 Jan 28.

            ●● Enlace al texto completo (gratuito o de pago) 1200/JCO.2012.47.0351

AUTORES / AUTHORS:  - Hadoux J; Ducreux M

INSTITUCIÓN / INSTITUTION:  - Institut Gustave Roussy, Villejuif, France.

 

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[13]

TÍTULO / TITLE:  - Merkel cell carcinoma arising in a patient with a history of multiple malignancies.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Cutis. 2012 Oct;90(4):183-5.

AUTORES / AUTHORS:  - Stevenson ML; Saggar S; Amin B; Mann RE

INSTITUCIÓN / INSTITUTION:  - Department of Dermatology, Columbia University School of Physicians and Surgeons, New York, New York, USA. marylstevenson@gmail.com

RESUMEN / SUMMARY:  - Merkel cell carcinoma (MCC) is a rare and highly aggressive neuroendocrine carcinoma of the skin. Although the association between MCC and other primary malignancies has been documented, the mechanism of this association has not been  elucidated. We report a case of MCC in a man with a history of multiple primary malignancies and treatment with immunomodulators. This case highlights the increased incidence of other malignancies in patients with MCC and is unique given the number and diversity of primary malignancies found in this patient.

 

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[14]

TÍTULO / TITLE:  - Diagnostic accuracy of an “amended” insulin-glucose ratio for the biochemical diagnosis of insulinomas.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Ann Intern Med. 2012 Dec 4;157(11):767-75. doi: 10.7326/0003-4819-157-11-201212040-00004.

            ●● Enlace al texto completo (gratuito o de pago) 7326/0003-4819-157-11-201212040-00004

AUTORES / AUTHORS:  - Nauck MA; Meier JJ

INSTITUCIÓN / INSTITUTION:  - Diabeteszentrum Bad Lauterberg, Kirchberg 21, D-37431 Bad Lauterberg im Harz, Germany. nauck@diabeteszentrum.de

RESUMEN / SUMMARY:  - BACKGROUND: Recent biochemical diagnostic guidelines for insulinomas require demonstration of hypoglycemia with inappropriately elevated (nonsuppressed) insulin, C-peptide, or proinsulin, but these criteria may overlap with those in patients without insulinomas. Use of an “amended” insulin-glucose ratio that accounts for the normal variation in insulin secretion according to prevailing glycemia may improve diagnostic accuracy. OBJECTIVE: To compare the diagnostic accuracy of current diagnostic guideline criteria with the amended insulin-glucose ratio in patients with a suspected insulinoma. DESIGN: Retrospective cohort study. SETTING: 2 specialized university departments in Germany. PATIENTS: 114 patients with suspected hypoglycemia over 10 years having  diagnostic prolonged fasts. MEASUREMENTS: Glucose, insulin, C-peptide, and the amended insulin-glucose ratio were measured during and at discontinuation of prolonged fasts. RESULTS: Of 114 patients who were evaluated, 49 had surgical resection of histologically confirmed insulinomas. Insulinoma was excluded in 65  patients; follow-up for a mean of 10 years (range, 0 to 16 years) showed no progressively severe hypoglycemic events or diagnoses of insulinoma. Patients with insulinoma had lower glucose levels and higher insulin and C-peptide levels  overall than did control patients at the end of prolonged fasts, but there was considerable overlap. The amended insulin-glucose ratio correctly identified 48 of 49 patients with insulinoma and excluded the diagnosis in 64 of 65 control patients, resulting in positive and negative predictive values of 0.98 (95% CI, 0.89 to 1.00) and 0.99 (CI, 0.92 to 1.00), respectively, compared with 0.75 (CI,  0.63 to 0.85) and 0.98 (CI, 0.89 to 1.00), respectively, for glucose, insulin, and C-peptide concentration criteria. LIMITATION: The study had a retrospective design, no proinsulin concentrations were available, and a nonspecific insulin immunoassay (crossreactive with proinsulin) was used. CONCLUSION: The amended insulin-glucose ratio showed improved diagnostic accuracy over established criteria that use glucose, insulin, and C-peptide concentrations. PRIMARY FUNDING SOURCE: None.

 

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[15]

TÍTULO / TITLE:  - Novel HIF2A mutations disrupt oxygen sensing leading to polycythemia, paragangliomas and somatostatinomas.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Blood. 2013 Jan 29.

            ●● Enlace al texto completo (gratuito o de pago) 1182/blood-2012-10-460972

AUTORES / AUTHORS:  - Yang C; Sun MG; Matro J; Huynh TT; Rahimpour S; Prchal JT; Lechan R; Lonser R; Pacak K; Zhuang Z

INSTITUCIÓN / INSTITUTION:  - Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, United States;

RESUMEN / SUMMARY:  - Hypoxia inducible factors (HIFs) control the cellular response to hypoxia and when dysregulated, contribute to tumorigenesis. Previously, we identified two gain-of-function somatic mutations in patients presenting with multiple paragangliomas or somatostatinomas, and polycythemia. Here we report two additional unique HIF2A mutations, which disrupt the hydroxylation domain recognized by prolyl hydroxylase domain-2 containing protein (PHD2), leading to stabilization of HIF-2alpha and increased expression of hypoxia-related genes.

 

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[16]

TÍTULO / TITLE:  - The Impact of Preradiation Residual Disease Volume on Time to Locoregional Failure in Cutaneous Merkel Cell Carcinoma-A TROG Substudy.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Int J Radiat Oncol Biol Phys. 2013 Jan 3. pii: S0360-3016(12)03824-2. doi: 10.1016/j.ijrobp.2012.11.026.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.ijrobp.2012.11.026

AUTORES / AUTHORS:  - Finnigan R; Hruby G; Wratten C; Keller J; Tripcony L; Dickie G; Rischin D; Poulsen M

INSTITUCIÓN / INSTITUTION:  - Division of Cancer Services, Princess Alexandra Hospital, University of Queensland, Brisbane, Australia.

RESUMEN / SUMMARY:  - PURPOSE: This study evaluated the impact of margin status and gross residual disease in patients treated with chemoradiation therapy for high-risk stage I and II Merkel cell cancer (MCC). METHODS AND MATERIALS: Data were pooled from 3 prospective trials in which patients were treated with 50 Gy in 25 fractions to the primary lesion and draining lymph nodes and 2 schedules of carboplatin based  chemotherapy. Time to locoregional failure was analyzed according to the burden of disease at the time of radiation therapy, comparing patients with negative margins, involved margins, or macroscopic disease. RESULTS: Analysis was performed on 88 patients, of whom 9 had microscopically positive resection margins and 26 had macroscopic residual disease. The majority of gross disease was confined to nodal regions. The 5-year time to locoregional failure, time to distant failure, time to progression, and disease-specific survival rates for the whole group were 73%, 69%, 62%, and 66% respectively. The hazard ratio for macroscopic disease at the primary site or the nodes was 1.25 (95% confidence interval 0.57-2.77), P=.58. CONCLUSIONS: No statistically significant differences in time to locoregional failure were identified between patients with negative margins and those with microscopic or gross residual disease. These results must, however, be interpreted with caution because of the limited sample size.

 

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[17]

TÍTULO / TITLE:  - Phase II study of sunitinib in Japanese patients with unresectable or metastatic, well-differentiated pancreatic neuroendocrine tumor.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Invest New Drugs. 2012 Dec 27.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s10637-012-9910-y

AUTORES / AUTHORS:  - Ito T; Okusaka T; Nishida T; Yamao K; Igarashi H; Morizane C; Kondo S; Mizuno N; Hara K; Sawaki A; Hashigaki S; Kimura N; Murakami M; Ohki E; Chao RC; Imamura M

INSTITUCIÓN / INSTITUTION:  - Department of Medicine and Bioregulatory Science, Graduate School of Medical Science, Kyushu University, 3-1-1 Maidashi Higashi-ku, Fukuoka, Japan, itopapa@intmed3.med.kyushu-u.ac.jp.

RESUMEN / SUMMARY:  - Background. Pancreatic neuroendocrine tumors (NETs) are rare but are frequently diagnosed at advanced stages and require systemic therapy. Patients and methods.  This multicenter, open-label, phase II study evaluated sunitinib in Japanese patients with well-differentiated pancreatic NET. Patients received sunitinib 37.5 mg/day on a continuous daily dosing (CDD) schedule. The primary endpoint was clinical benefit rate (CBR; percentage of complete responses [CRs] plus partial responses [PRs] plus stable disease [SD] >/=24 weeks). Secondary endpoints included objective response rate (ORR), tumor shrinkage, progression-free survival (PFS) probability, safety, pharmacokinetics, and biomarkers. Results. Twelve patients received treatment. The CBR was 75 % (95 % confidence interval [CI], 43-94) and included 6 patients with a PR and 3 with SD. The ORR was 50 % (95 % CI, 21-79). PFS probability was 91 % (95 % CI, 54-99) at 6 months and 71 %  (95 % CI, 34-90) at 12 months. Commonly reported treatment-emergent (all-causality), any-grade adverse events included diarrhea (n = 10), hand-foot syndrome and hypertension (both n = 8), fatigue and headache (both n = 7), and neutropenia (n = 6). No deaths on study were reported; one death due to disease progression occurred >28 days after end of treatment. Sunitinib on a CDD schedule resulted in sustained drug concentrations without accumulation across cycles. Tumor responses in all 12 patients did not appear to correlate with decreases in  chromogranin A levels. Conclusions. Sunitinib 37.5 mg/day on a CDD schedule demonstrated antitumor activity in Japanese patients with unresectable, well-differentiated pancreatic NET. Commonly reported adverse events were consistent with the known safety profile of sunitinib.

 

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[18]

TÍTULO / TITLE:  - Are neuroendocrine tumors going mainstream?

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Clin Oncol. 2013 Feb 1;31(4):404-5. doi: 10.1200/JCO.2012.47.3884. Epub 2012 Dec 17.

            ●● Enlace al texto completo (gratuito o de pago) 1200/JCO.2012.47.3884

AUTORES / AUTHORS:  - Kulke MH

INSTITUCIÓN / INSTITUTION:  - Department of Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Ave,  Boston, MA 02115; matthew_kulke@dfci.harvard.edu.

 

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[19]

TÍTULO / TITLE:  - Echo rounds: undiagnosed severe pulmonic regurgitation in a carcinoid patient for tricuspid valve replacement and patent foramen ovale closure.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Anesth Analg. 2013 Feb;116(2):300-2. doi: 10.1213/ANE.0b013e3182753538. Epub 2013 Jan 9.

            ●● Enlace al texto completo (gratuito o de pago) 1213/ANE.0b013e3182753538

AUTORES / AUTHORS:  - Hastie MJ; Souh J; Ju A; Shanewise JS

INSTITUCIÓN / INSTITUTION:  - Department of Anesthesiology, Division of Adult Cardiothoracic Anesthesiology, Columbia University, 622 W 168th street, PH 5, Rm505, New York, NY 10032. mj2081@columbia.ed.

 

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[20]

TÍTULO / TITLE:  - Expression of the coxsackie adenovirus receptor in neuroendocrine lung cancers and its implications for oncolytic adenoviral infection.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Cancer Gene Ther. 2013 Jan;20(1):25-32. doi: 10.1038/cgt.2012.80. Epub 2012 Nov 30.

            ●● Enlace al texto completo (gratuito o de pago) 1038/cgt.2012.80

AUTORES / AUTHORS:  - Wunder T; Schmid K; Wicklein D; Groitl P; Dobner T; Lange T; Anders M; Schumacher U

INSTITUCIÓN / INSTITUTION:  - Department of Anatomy and Experimental Morphology, University Hospital Hamburg-Eppendorf, Hamburg, Germany.

RESUMEN / SUMMARY:  - Coxsackie adenovirus receptor (CAR) is the primary receptor to which oncolytic adenoviruses have to bind for internalization and viral replication. A total of 171 neuroendocrine lung tumors in form of multitissue arrays have been analyzed resulting in a positivity of 112 cases (65.5%). Immunostaining correlated statistically significant with histopathology and development of recurrence. The  subtype small cell lung cancer (SCLC) showed the highest CAR expression (77.6%),  moreover the CAR level was correlated to the disease-free survival. Further, high CAR expression level in SCLC cell lines was found in vitro and in vivo when cell  lines had been transplanted into immunodeficient mice. A correlation between CAR  expression in the primary tumors and metastases development in the tumor model underlined the clinical relevance. Cell lines with high CAR level showed a high infectivity when infected with a replication-deficient adenovirus. Low levels of  CAR expression in SCLC could be upregulated with Trichostatin A, a histone deacetylase inhibitor. As a result of the unaltered poor prognosis of SCLC and its high CAR expression it seems to be the perfect candidate for oncolytic therapy. With our clinically relevant tumor model, we show that xenograft experiments are warrant to test the efficiency of oncolytic adenoviral therapy.

 

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[21]

TÍTULO / TITLE:  - Distinct Gene Expression Profiles of Viral- and Nonviral-Associated Merkel Cell Carcinoma Revealed by Transcriptome Analysis.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Invest Dermatol. 2012 Dec 6. doi: 10.1038/jid.2012.445.

            ●● Enlace al texto completo (gratuito o de pago) 1038/jid.2012.445

AUTORES / AUTHORS:  - Harms PW; Patel RM; Verhaegen ME; Giordano TJ; Nash KT; Johnson CN; Daignault S; Thomas DG; Gudjonsson JE; Elder JT; Dlugosz AA; Johnson TM; Fullen DR; Bichakjian CK

INSTITUCIÓN / INSTITUTION:  - 1] Department of Pathology, University of Michigan Medical Center, Ann Arbor, Michigan, USA [2] Department of Dermatology, University of Michigan Medical Center, Ann Arbor, Michigan, USA.

RESUMEN / SUMMARY:  - Merkel cell carcinoma (MCC) is an aggressive cutaneous neuroendocrine tumor with  high mortality rates. Merkel cell polyomavirus (MCPyV), identified in the majority of MCCs, may drive tumorigenesis via viral T antigens. However, the mechanisms underlying pathogenesis in MCPyV-negative MCCs remain poorly understood. To nominate genes contributing to the pathogenesis of MCPyV-negative  MCCs, we performed DNA microarray analysis on 30 MCCs. The MCPyV status of MCCs was determined by PCR for viral DNA and RNA. A total of 1,593 probe sets were differentially expressed between MCPyV-negative and MCPyV-positive MCCs, with significant differential expression defined as at least a 2-fold change in either direction and a P-value </=0.05. MCPyV-negative tumors showed decreased RB1 expression, whereas MCPyV-positive tumors were enriched for immune response genes. Validation studies included immunohistochemistry demonstration of decreased RB protein expression in MCPyV-negative tumors and increased peritumoral CD8+ T lymphocytes surrounding MCPyV-positive tumors. In conclusion,  our data suggest that loss of RB1 expression may have an important role in the tumorigenesis of MCPyV-negative MCCs. Functional and clinical validation studies  are needed to determine whether this tumor-suppressor pathway represents an avenue for targeted therapy.Journal of Investigative Dermatology advance online publication, 6 December 2012; doi:10.1038/jid.2012.445.

 

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[22]

TÍTULO / TITLE:  - Long-term outcomes of surgical treatment for hereditary pheochromocytoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Am Coll Surg. 2013 Feb;216(2):280-9. doi: 10.1016/j.jamcollsurg.2012.10.012.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.jamcollsurg.2012.10.012

AUTORES / AUTHORS:  - Grubbs EG; Rich TA; Ng C; Bhosale PR; Jimenez C; Evans DB; Lee JE; Perrier ND

INSTITUCIÓN / INSTITUTION:  - Department of Surgical Oncology, University of Texas MD Anderson Cancer Center, Houston, TX. Electronic address: eggrubbs@mdanderson.org.

RESUMEN / SUMMARY:  - BACKGROUND: The ideal surgical management of hereditary pheochromocytomas includes planning for a potential metachronous bilateral presentation and the possibility of lifelong steroid dependence if bilateral adrenalectomy is needed.  An intact and viable cortical remnant after bilateral pheochromocytoma resection  can eliminate the necessity for steroid dependency, but can increase the risk of  pheochromocytoma recurrence. STUDY DESIGN: We retrospectively reviewed outcomes of all patients with a diagnosis of hereditary pheochromocytomas treated at our tertiary cancer institution from 1962-2011, with subset analysis of patients undergoing a cortical-sparing procedure in the setting of bilateral adrenalectomy. RESULTS: Of the ninety-six patients who underwent adrenalectomy for hereditary pheochromocytomas, 47 presented with bilateral disease. In 15 of the 49 patients (30%) who originally underwent unilateral adrenalectomy, pheochromocytoma developed in the contralateral gland at a median of 8.2 years (range 1 to 20 years) after the initial diagnosis. There were 4 recurrences in 55 cortical-sparing remnants (7%) and 3 recurrences in the adrenal bed after 101 intended total adrenal resections (3%) (p = 0.24). Total bilateral adrenalectomy  was performed in 25 patients and acute adrenal insufficiency developed in 5 (20%) of those patients. An intended cortical-sparing adrenalectomy was performed in 39 patients and acute adrenal insufficiency developed in 1 (3%). Of these patients with adequate follow-up, 21 of 27 (78%) were steroid independent at 3-year follow-up. Sex, median age, adrenal vein preservation, metachronous adrenal resection, and bilateral cortical-sparing procedures did not predict steroid independence at 3 years. CONCLUSIONS: Cortical-sparing adrenalectomy avoids long-term corticosteroid dependence in the majority of patients with hereditary pheochromocytoma with minimal risk of acute adrenal insufficiency. Recurrence occurs in approximately 7% of adrenal remnants.

 

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[23]

TÍTULO / TITLE:  - Comparison of serum calcitonin and procalcitonin in detecting medullary thyroid carcinoma among patients with thyroid nodules.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Clin Chem. Acceso gratuito al texto completo a partir de 1 año de la fecha de publicación.

            ●● Enlace a la Editora de la Revista http://www.journals.uchicago.edu/ 

            ●● Cita: Clinical Infectious Diseases: <> Lab Med. 2012 Dec 25:1-5. doi: 10.1515/cclm-2012-0610.

            ●● Enlace al texto completo (gratuito o de pago) 1515/cclm-2012-0610

AUTORES / AUTHORS:  - Giovanella L; Verburg FA; Imperiali M; Valabrega S; Trimboli P; Ceriani L

RESUMEN / SUMMARY:  - Abstract Background: To prospectively evaluate the role of procalcitonin (PCT) in detecting or excluding medullary thyroid carcinoma (MTC) among patients with thyroid nodules and increased calcitonin (CT) levels. Methods: Fourteen of 1236 patients referred for thyroid nodules had increased serum CT >10 pg/mL. A stimulation test with pentagastrin was done and both CT and PCT were measured after stimulation. All patients underwent thyroid ultrasound, fine-needle cytology and, if indicated, surgery with histological and immunohistochemical examination of the surgical specimens. Results: After follow-up, two MTCs were found. These two patients had basal CT >100 pg/mL and detectable (>0.1 ng/mL) PCT, with 100% sensitivity. Pentagastrin stimulated CT achieved values above 100  pg/mL in two MTCs and in other two cases with no MTC outcome (50% PPV and 83% NPV). On the contrary, all patients with no MTC had both basal and stimulated undetectable PCT (100% PPV and 100% NPV). Conclusions: The addition of basal PCT  measurement in patients with thyroid nodule(s) and increased CT may significantly improve accuracy of CT measurement without needing a PG stimulation test.

 

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[24]

TÍTULO / TITLE:  - Axl/Gas6/NFkappaB signalling in schwannoma pathological proliferation, adhesion and survival.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Oncogene. 2013 Jan 14. doi: 10.1038/onc.2012.587.

            ●● Enlace al texto completo (gratuito o de pago) 1038/onc.2012.587

AUTORES / AUTHORS:  - Ammoun S; Provenzano L; Zhou L; Barczyk M; Evans K; Hilton DA; Hafizi S; Hanemann CO

INSTITUCIÓN / INSTITUTION:  - Clinical Neurobiology, Institute of Translational and Stratified Medicine, Plymouth University Peninsula Schools of Medicine and Dentistry, Plymouth, UK.

RESUMEN / SUMMARY:  - TAM family receptor tyrosine kinases comprising Tyro3 (Sky), Axl, and Mer are overexpressed in some cancers, correlate with multidrug resistance and contribute to tumourigenesis by regulating invasion, angiogenesis, cell survival and tumour  growth. Mutations in the gene coding for a tumour suppressor merlin cause development of multiple tumours of the nervous system such as schwannomas, meningiomas and ependymomas occurring spontaneously or as part of a hereditary disease neurofibromatosis type 2. The benign character of merlin-deficient tumours makes them less responsive to chemotherapy. We previously showed that, amongst other growth factor receptors, TAM family receptors (Tyro3, Axl and Mer)  are significantly overexpressed in schwannoma tissues. As Axl is negatively regulated by merlin and positively regulated by E3 ubiquitin ligase CRL4DCAF1, previously shown to be a key regulator in schwannoma growth we hypothesized that  Axl is a good target to study in merlin-deficient tumours. Moreover, Axl positively regulates the oncogene Yes-associated protein, which is known to be under merlin regulation in schwannoma and is involved in increased proliferation  of merlin-deficient meningioma and mesothelioma. Here, we demonstrated strong overexpression and activation of Axl receptor as well as its ligand Gas6 in human schwannoma primary cells compared to normal Schwann cells. We show that Gas6 is mitogenic and increases schwannoma cell-matrix adhesion and survival acting via Axl in schwannoma cells. Stimulation of the Gas6/Axl signalling pathway recruits  Src, focal adhesion kinase (FAK) and NFkappaB. We showed that NFkappaB mediates Gas6/Axl-mediated overexpression of survivin, cyclin D1 and FAK, leading to enhanced survival, cell-matrix adhesion and proliferation of schwannoma. We conclude that Axl/FAK/Src/NFkappaB pathway is relevant in merlin-deficient tumours and is a potential therapeutic target for schwannoma and other merlin-deficient tumours.Oncogene advance online publication, 14 January 2013; doi:10.1038/onc.2012.587.

 

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[25]

TÍTULO / TITLE:  - Vandetanib for the Treatment of Medullary Thyroid Cancer.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Clin Cancer Res. 2013 Jan 22.

            ●● Enlace al texto completo (gratuito o de pago) 1158/1078-0432.CCR-12-2353

AUTORES / AUTHORS:  - Chau NG; Haddad RI

INSTITUCIÓN / INSTITUTION:  - Authors’ Affiliation: Medical Oncology, Head and Neck Oncology Program, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts.

RESUMEN / SUMMARY:  - Vandetanib (ZD6474, Caprelsa, AstraZeneca), an oral small-molecule tyrosine kinase inhibitor (TKI) that targets the rearranged during transfection receptor (RET), VEGF receptor (VEGFR2-3), and EGF receptor (EGFR), is the first systemic therapy approved by the U.S. Food and Drug Administration (FDA) for the treatment of symptomatic or progressive advanced medullary thyroid cancer (MTC). In a randomized phase III trial of patients with unresectable, locally advanced, or metastatic MTC, vandetanib improved progression-free survival compared with placebo [HR, 0.46; 95% confidence interval (CI), 0.31-0.69; P < 0.001]. However,  the benefits in delaying disease progression need to be balanced against the associated and potentially serious toxicities, including diarrhea, hypertension,  and QTc prolongation. Here, we review the clinical development of vandetanib leading to its integration into the current treatment paradigm and highlight the  ongoing and future challenges in TKI use in MTC. Clin Cancer Res; 19(3); 1-6. ©2012 AACR.

 

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[26]

TÍTULO / TITLE:  - Correlation between a short-term intravenous octreotide suppression test and response to antrectomy in patients with type-1 gastric neuroendocrine tumours.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Eur J Gastroenterol Hepatol. 2012 Dec 16.

            ●● Enlace al texto completo (gratuito o de pago) 1097/MEG.0b013e32835cec52

AUTORES / AUTHORS:  - Murugesan SV; Steele IA; Dimaline R; Poston GJ; Shrotri M; Campbell F; Varro A; Pritchard DM

INSTITUCIÓN / INSTITUTION:  - Departments of aGastroenterology bPhysiology, Institute of Translational Medicine, University of Liverpool cDepartment of Surgery, Aintree University Hospital NHS Foundation Trust dDepartment of Pathology, Royal Liverpool and Broadgreen University Hospitals NHS Trust, Liverpool, UK.

RESUMEN / SUMMARY:  - BACKGROUND: Type-1 gastric neuroendocrine tumours (NETs) arise in some patients with chronic hypergastrinaemia secondary to autoimmune atrophic gastritis. Patients with small tumours are usually managed conservatively, because their prognosis is very good. However, larger tumours may require surgical intervention. Many type-1 gastric NETs regress following antrectomy because this  removes the source of hypergastrinaemia. However, some tumours do not regress following antrectomy and additional surgery may be required. An octreotide suppression test has been previously suggested as a means to assess whether type-1 gastric NETs are likely to regress following antrectomy. AIM: To prospectively examine the role of a short-term intravenous octreotide suppression test in predicting type-1 gastric NET regression in five patients who subsequently underwent antrectomy. MATERIALS AND METHODS: Serum gastrin concentrations and gastric corpus and tumour histidine decarboxylase mRNA abundances were assessed in patients with type-1 gastric NETs before and 72 h after the administration of 25 microg/h intravenous octreotide. Gastric tumour response was assessed endoscopically following subsequent antrectomy. RESULTS: All patients showed significant decreases in serum gastrin concentrations as well as corpus and tumour biopsy histidine decarboxylase mRNA abundance following octreotide infusion. All patients also showed resolution of hypergastrinaemia following subsequent antrectomy. However, tumour regression was only observed in  four of the five patients. One patient had a persistent tumour 3 years after antrectomy and required additional surgical resection. CONCLUSION: A positive octreotide suppression test result does not always predict response to antrectomy in patients with type-1 gastric NETs. Assessment of gastric mucosal responses to  a gastrin/CCK-2 receptor antagonist may therefore also be helpful.

 

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[27]

TÍTULO / TITLE:  - Treatment, prognostic markers and survival in thymic neuroendocrine tumours. A study from a single tertiary referral centre.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Lung Cancer. 2012 Dec 31. pii: S0169-5002(12)00646-0. doi: 10.1016/j.lungcan.2012.12.001.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.lungcan.2012.12.001

AUTORES / AUTHORS:  - Crona J; Bjorklund P; Welin S; Kozlovacki G; Oberg K; Granberg D

INSTITUCIÓN / INSTITUTION:  - Experimental Surgery, Department of Surgical Sciences, Uppsala University, S-751  85, Uppsala, Sweden. Electronic address: Joakim.Crona@surgsci.uu.se.

RESUMEN / SUMMARY:  - Thymic neuroendocrine tumours (TNETs) are uncommon but malignant neoplasms, usually associated with a poor prognosis. The number of cases reported is limited to a few hundreds and there are few prognostic factors available. All 28 patients (22 male, 6 female; median age 46.5 years) with thymic neuroendocrine tumour, treated at the Department of Endocrine Oncology, Uppsala University Hospital, Uppsala, Sweden between 1985 and 2011 were studied. The overall 3, 5 and 10-year  survival was 89%, 79% and 41% respectively. Ki67<10% (p=0.018) as well as surgical resection (p=0.001) and macroscopically radical primary surgery (p=0.034) was associated with increased survival. Staging & grading according to  Masaoka and ENETS systems did not correlate with survival. However, a modified ENETS grading showed a positive correlation (p=0.015). Median time to progression was 20.5 months with Temozolomide and 18 months with platinum based therapy. Partial responses were noted in three patients (38%) treated with platinum based  therapy and in two patients (20%) treated with Temozolomide based therapy. High proliferative rate, measured by Ki67 index, and absence of macroscopically radical primary resection as well as no surgical resection are three negative prognostic factors in patients with TNETs. Temozolomide or Platinum based chemotherapy should be considered as first-line medical therapy in patients with  metastatic or non-resectable tumours.

 

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[28]

TÍTULO / TITLE:  - Anti-tumor effects of peptide analogs targeting neuropeptide hormone receptors on mouse pheochromocytoma cells.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Mol Cell Endocrinol. 2012 Dec 23. pii: S0303-7207(12)00535-7. doi: 10.1016/j.mce.2012.12.011.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.mce.2012.12.011

AUTORES / AUTHORS:  - Ziegler CG; Ullrich M; Schally AV; Bergmann R; Pietzsch J; Gebauer L; Gondek K; Qin N; Pacak K; Ehrhart-Bornstein M; Eisenhofer G; Bornstein SR

INSTITUCIÓN / INSTITUTION:  - University Hospital Carl Gustav Carus, Department of Medicine III, Dresden, Germany. Electronic address: Christian.Ziegler@uniklinikum-dresden.de.

RESUMEN / SUMMARY:  - Pheochromocytoma is a rare but potentially lethal chromaffin cell tumor with currently no effective treatment. Peptide hormone receptors are frequently overexpressed on endocrine tumor cells and can be specifically targeted by various anti-tumor peptide analogs. The present study carried out on mouse pheochromocytoma cells (MPCs) and a more aggressive mouse tumor tissue-derived (MTT) cell line revealed that these cells are characterized by pronounced expression of the somatostatin receptor 2 (sst2), growth hormone-releasing hormone (GHRH) receptor and the luteinizing hormone-releasing hormone (LHRH) receptor. We further demonstrated significant anti-tumor effects mediated by cytotoxic somatostatin analogs, AN-162 and AN-238, by LHRH antagonist, Cetrorelix, by the cytotoxic LHRH analog, AN-152, and by recently developed GHRH  antagonist, MIA-602, on MPC and for AN-152 and MIA-602 on MTT cells. Studies of novel anti-tumor compounds on these mouse cell lines serve as an important basis  for mouse models of metastatic pheochromocytoma, which we are currently establishing.

 

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[29]

TÍTULO / TITLE:  - Necrolytic migratory erythema in a patient with neuroendocrine carcinoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Intern Med. 2013;52(1):151-2. Epub 2013 Jan 1.

AUTORES / AUTHORS:  - Gronau M; Jager D; Enk AH; Hassel JC

INSTITUCIÓN / INSTITUTION:  - National Center for Tumor Diseases Heidelberg, Germany.

RESUMEN / SUMMARY:  - Necrolytic migratory erythema is an obligatory paraneoplastic syndrome. Here we describe a patient with a neuroendocrine tumor of probably pancreatic origin. She developed the typical skin lesions under chemotherapy with temozolomide and capecitabine.

 

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[30]

TÍTULO / TITLE:  - Thioredoxin-Mimetic Peptides (TXM) Reverse Auranofin Induced Apoptosis and Restore Insulin Secretion in Insulinoma Cells.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Biochem Pharmacol. 2013 Jan 14. pii: S0006-2952(13)00020-8. doi: 10.1016/j.bcp.2013.01.003.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.bcp.2013.01.003

AUTORES / AUTHORS:  - Cohen-Kutner M; Khomsky L; Trus M; Aisner Y; Niv MY; Benhar M; Atlas D

INSTITUCIÓN / INSTITUTION:  - Department of Biological Chemistry, The Institute of Life Sciences, The Hebrew University of Jerusalem, Jerusalem, 919104, Israel.

RESUMEN / SUMMARY:  - The thioredoxin reductase/thioredoxin system (TrxR/Trx1) plays a major role in protecting cells from oxidative stress. Disruption of the TrxR-Trx1 system keeps  Trx1 in the oxidized state leading to cell death through activation of the ASK1-Trx1 apoptotic pathway. The potential mechanism and ability of tri- and tetra oligopeptides derived from the canonical -CxxC- motif of the Trx1-active site to mimic and enhance Trx1 cellular activity, was examined. The Trx mimetics  peptides (TXM) protected insulinoma INS832/13 cells from oxidative stress induced by selectively inhibiting TrxR with auranofin (AuF). TXM reversed the AuF-effects preventing apoptosis, and increasing cell-viability. The TXM peptides were effective in inhibiting AuF-induced MAPK, JNK and p38(MAPK) phosphorylation, in correlation with preventing caspase-3 cleavage and thereby PARP-1 dissociation. The ability to form a disulfide-bridge-like conformation was estimated from molecular dynamics simulations. The TXM peptides restored insulin secretion and displayed Trx1 denitrosylase activity. Their potency was 10 to 100 fold higher than redox reagents like NAC, AD4, or ascorbic acid. Unable to reverse ERK1/2 phosphorylation, TXM-CB3 (NAc-Cys-Pro-Cys amide) appeared to function in part, through inhibiting ASK1-Trx dissociation. These highly effective anti-apoptotic effects of Trx1 mimetic peptides exhibited in INS832/13 cells could become valuable in treating adverse oxidative-stress related disorders such as diabetes.

 

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[31]

TÍTULO / TITLE:  - The presence of merkel cell polyomavirus is associated with deregulated expression of BRAF and Bcl-2 genes in non-small cell lung cancer.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Int J Cancer. 2013 Jan 25. doi: 10.1002/ijc.28062.

            ●● Enlace al texto completo (gratuito o de pago) 1002/ijc.28062

AUTORES / AUTHORS:  - Lasithiotaki I; Antoniou KM; Derdas SP; Sarchianaki E; Symvoulakis EK; Psaraki A; Spandidos DA; Stathopoulos EN; Siafakas NM; Sourvinos G

INSTITUCIÓN / INSTITUTION:  - Department of Thoracic Medicine, University Hospital, Medical School, University  of Crete, Heraklion 71110, Crete.

RESUMEN / SUMMARY:  - Polyomaviruses such as BK virus (BKV), JC virus (JCV) and Merkel Cell Polyomavirus (MCPyV) are typically non-oncogenic, although they have been detected in a variety of human neoplasms. The aim of this study was to determine  the frequency of the most common polyomaviruses MCPyV, BKV and JCV as well as the gene expression profile of genes involved in oncogenesis including K-ras, BRAF, RKIP, Bax, Bcl-2, p53 and RB1 in a cohort of non-small cell lung cancer (NSCLC) patients. Real-Time and nested PCR were employed to assess the presence of polyomaviruses DNA in tissue biopsies from 110 patients with primary NSCLC and 14 tissue specimens from macroscopically healthy sites of their lung. Real Time PCR  was also employed to determine the mRNA expression of K-ras, BRAF, RKIP, Bax, Bcl-2, p53 and RB1 in selected samples. Results showed that 10 NSCLC specimens were positive for the presence of MCPyV DNA (10/110, 9.1%), whereas no control sample was tested positive for the virus. The MCPyV-positive samples were predominantly obtained from male smokers (9/10). BKV and JCV DNA was not detected either in lung tissues biopsies or the control specimens. Interestingly, gene expression analysis revealed increased mRNA and protein expression of BRAF gene in association with BRAF phosphorylation in the MCPyV-positive samples, whereas Bcl-2 gene expression was downregulated in the same type of samples. The detected MCPyV prevalence in NSCLC in combination with the deregulated expression of BRAF  and Bcl-2 genes suggests that these events are likely to contribute to the pathogenesis of NSCLC.

 

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[32]

TÍTULO / TITLE:  - RET proto-oncogene genetic screening of families with multiple endocrine neoplasia type 2 optimizes diagnostic and clinical management in China.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Thyroid. 2012 Dec;22(12):1257-65. doi: 10.1089/thy.2012.0134.

            ●● Enlace al texto completo (gratuito o de pago) 1089/thy.2012.0134

AUTORES / AUTHORS:  - Qi XP; Chen XL; Ma JM; Du ZF; Fei J; Yang CP; Cheng J; Song QZ; Han JS; Jin HY; Chen ZG; Wang JQ; Yang YP; Ying RB; Liu WT; Zhao Y; Chen CY; Jiang HL; Ke HP; Zhang XN

INSTITUCIÓN / INSTITUTION:  - Department of Oncologic Surgery, The 117th PLA Hospital, Hangzhou, China. qxplmd@163.com

RESUMEN / SUMMARY:  - BACKGROUND: Genetic screening for germline mutations in the RET proto-oncogene has been extensively exploited worldwide to optimize the diagnostic and clinical  management of multiple endocrine neoplasia type 2 (MEN2) patients and their relatives. However, a distinct lag period exists not only in the recognition but  also in the medical treatment of patients with MEN2. Here we present a comprehensive genetic and clinical analysis of MEN2 among Chinese families followed from 1975 to 2011. Our series comprises 36 index cases and 134 relatives from 11 independent families. METHODS: Genetic diagnosis was performed in all participants by direct sequencing all relevant RET exons. Thyroidectomy was performed in 50 patients with varying cervical neck dissection procedures. Patients with pheochromocytoma (PHEO) underwent specific surgery. Demographic, clinical profiles, mutation types, tumor histopathologic features, and follow-up  records were systematically analyzed. RESULTS: The RET mutations p.C634Y (n=34),  p.C634R (n=6), p.C618S (n=13), p.V292M/R67H/R982C (n=7), p.L790F (n=2), and p.C634Y/V292M/R67H/R982C (n=1) were confirmed in 31 index cases and then identified in 32 at-risk relatives (mutation carriers), with MEN2A as the most common clinical subtype. The overall penetrance of PHEO in patients with MEN2A was 46.7%. A total of 50 patients underwent thyroidectomy, and there was a significant lowering of their mean age at thyroidectomy and the tumor diameter of the mutation carriers that were detected and operated on compared with the index  cases (age at first surgery: 29.3 vs. 39.3 years, p<0.05; maximum size: 1.1 vs. 3.3 cm, p<0.001). There was also a decrease in the TNM staging and the proportion of patients who underwent inappropriate initial thyroid surgery (pN1: 31.6% vs. 100%, p<0.001; inappropriate surgery: 0% vs. 29%). Meanwhile, disease-free survival (DFS) increased (DFS: 100% vs. 58.1%, p<0.05). Both medullary thyroid carcinoma-specific (n=1) and PHEO-specific (n=5) deaths were reported during the  study period. CONCLUSIONS: Our results further substantiate that gene scanning of all relevant RET exons is a powerful tool in the management of MEN2 patients, especially in asymptomatic carriers, and has led to earlier diagnosis and more complete initial treatment of patients with MEN2 in China.

 

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[33]

TÍTULO / TITLE:  - Genes of detoxification are important modulators of hereditary medullary thyroid  carcinoma risk.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Clin Endocrinol (Oxf). 2012 Dec 24. doi: 10.1111/cen.12136.

            ●● Enlace al texto completo (gratuito o de pago) 1111/cen.12136

AUTORES / AUTHORS:  - Barbieri RB; Bufalo NE; Cunha LL; Assumpcao LV; Maciel RM; Cerutti JM; Ward LS

INSTITUCIÓN / INSTITUTION:  - Laboratory of Cancer Molecular Genetics, Faculty of Medical Sciences, University  of Campinas (FCM-Unicamp), Campinas, SP, Brazil.

RESUMEN / SUMMARY:  - CONTEXT: Different inherited profiles of genes involved in cellular mechanisms of activation and detoxification of carcinogenic products can provide specific protection or determine the risk for cancer. Low-penetrance polymorphic genes related to the biotransformation of environmental toxins have been associated with susceptibility to, and the phenotype of, human tumours. OBJECTIVE: To investigate the role of germline inheritance of polymorphisms in CYP1A2*F, CYP1A1m1, GSTP1, NAT2 and TP53 genes in hereditary medullary thyroid carcinoma (HMTC) patients. DESIGN: This study was developed in University of Campinas (Unicamp). PATIENTS: We studied 132 patients with HMTC, 88 first-degree relatives of HMTC patients and 575 control individuals. MEASUREMENTS: All patients with MTC and their relatives were sequenced for the RET gene and five genes were genotyped using TaqMan(®) system. RESULTS: We observed that the inheritance of CYP1A2*F (OR=2.10; 95%CI=1.11 to 3.97; p=0.022), GSTP1 (OR=4.41; 95%CI=2.47 to 7.88; p<0.001) and NAT2 (OR=2.54; 95%CI=1.16 to 5.58; p=0.020) variants increased the risk for HMTC. In addition, multiple regression analysis showed that the inheritance of GSTP1 polymorphisms was associated with the diagnosis in older patients (B=8.0229; 95% IC= +/-5.5735; p=0.0054). Concerning the group of HTMC relatives, CYP1A2*F (OR=2:40; 95%CI=1.19-4.86; p=0.015), CYP1A1m1 (OR=2.79; 95% CI=1:04-7.51; p=0.042), GSTP1 (OR=2.86; IC 95%= 1.53-5.32; p<0.001) and NAT2 (OR=2.25; IC 95%= 1.20-4.22; p=0.012) were associated with HMTC risk. CONCLUSIONS: We have demonstrated that the inheritance of specific genes determining the individual response to environmental toxins may contribute to the risk and phenotypic variability that exists in patients with HMTC. Moreover, we identified a group at risk in relatives of HMTC patients. © 2012 Blackwell Publishing Ltd.

 

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[34]

TÍTULO / TITLE:  - Peak Incidence of Pheochromocytoma and Primary Hyperparathyroidism in Multiple Endocrine Neoplasia 2: Need for Age-Adjusted Biochemical Screening.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Clin Endocrinol Metab. 2013 Jan 2.

            ●● Enlace al texto completo (gratuito o de pago) 1210/jc.2012-3192

AUTORES / AUTHORS:  - Machens A; Lorenz K; Dralle H

INSTITUCIÓN / INSTITUTION:  - Department of General, Visceral, and Vascular Surgery, Martin Luther University Halle-Wittenberg, D-06097 Halle (Saale), Germany.

RESUMEN / SUMMARY:  - Context:In multiple endocrine neoplasia type 2, American Thyroid Association (ATA) management guidelines recommend continuous biochemical screening for pheochromocytoma and/or primary hyperparathyroidism. This implicit assumption of  linear tumor development is difficult to reconcile with current thinking that cells accrue somatic mutations stochastically, yielding a bell-shaped distribution.Objective:This investigation aimed at evaluating the age distribution of pheochromocytoma and primary hyperparathyroidism in gene carriers at risk of developing multiple endocrine neoplasia type 2.Design:ATA class D, C,  B, and A mutations, with or without pheochromocytoma and/or primary hyperparathyroidism, were plotted against carrier age at the time of diagnosis or last follow-up.Setting:The setting was a surgical referral center.Patients:Included were 474 carriers of ATA class D (37 patients), C (170 patients), B (112 patients), and A (155 patients) mutations. Eighty-four carriers (17.8%) developed pheochromocytoma (bilateral in 42 patients) and 20 carriers (4.2%) primary hyperparathyroidism.Interventions:Interventions were adrenalectomy and/or parathyroidectomy.Main Outcome Measures:Main outcome measures were multiple endocrine neoplasia type 2-associated tumors.Results:Bell-shaped age distribution curves were obtained for unilateral and bilateral pheochromocytoma (ATA class D, C, and B) and primary hyperparathyroidism (ATA class C and B). Owing to the rarity of events, the bell shape of the distribution curve was faint but consistent with a random distribution for ATA class A mutations (unilateral pheochromocytoma and primary hyperparathyroidism). With decreasing penetrance, the bell-shaped distribution curve, becoming narrower and flatter, shifted to the right toward higher age groups.Conclusions:These data, revealing phases of greater amidst phases of lower penetrance, support adjustment of biochemical screening to carrier age and ATA class.

 

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[35]

TÍTULO / TITLE:  - Enhanced neuronal differentiation of pheochromocytoma 12 cells on polydopamine-modified surface.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Biochem Biophys Res Commun. 2013 Jan 25;430(4):1294-300. doi: 10.1016/j.bbrc.2012.11.123. Epub 2012 Dec 19.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.bbrc.2012.11.123

AUTORES / AUTHORS:  - Bhang SH; Kwon SH; Lee S; Kim GC; Han AM; Kwon YH; Kim BS

INSTITUCIÓN / INSTITUTION:  - School of Chemical and Biological Engineering, Seoul National University, Seoul 151-744, Republic of Korea.

RESUMEN / SUMMARY:  - Since pheochromocytoma 12 (PC12) cells have the ability of neuronal differentiation upon nerve growth factor (NGF) treatment, they are a good model for studying the neuronal differentiation. Establishing a strong adhesion of PC12 cells to the culture substrate may increase neuronal differentiation, and the use of L-3,4-dihydroxyphenylalanine (L-DOPA), which is responsible for the adhesive property of mussel adhesive proteins (MAPs), is a feasible strategy for such strong adhesion. We hypothesized that a polydopamine-modified surface can promote PC12 cell adhesion and subsequent neuronal differentiation. We examined whether polydopamine-modified surface promotes PC12 cell adhesion, and further evaluated  the neuronal differentiation of these cells. The polydopamine modification enhanced the cell adhesion and viability, and also promoted the neuronal differentiation of NGF-stimulated PC12 cells, as evidenced by the elongation of neurites and expression of neuronal differentiation markers, by increasing the activation of NGF/Trk-Rho GTPase signal pathway. Our findings will help develop an improved strategy for functionalizing biomaterial substrates for less-adhesive cells including neural cells.

 

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[36]

TÍTULO / TITLE:  - Invasive neuroendocrine carcinoma of the breast: aprognostic research of 107 chinese patients.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Neoplasma. 2013;60(2):215-22.

AUTORES / AUTHORS:  - Zhang Y; Chen Z; Bao Y; DU Z; Li Q; Zhao Y; Tang F

RESUMEN / SUMMARY:  - Neuroendocrine carcinoma (NEC) of the breast, adistinct type of mammary carcinoma whose terminology was not proposed until 2003, has not been well recognized or studied. The aim of our study is to evaluate the clinicopathological features and outcomes of this type of tumor. We conducted acomparative study on 107 NEC patients and 475 invasive ductal carcinoma, not otherwise specified(IDC, NOS) patients from the Department of Pathology, Huashan Hospital, Fudan University, to determine the demographic, pathological, and clinical features at presentation, along with patient outcomes and prognostic factors. With an older age at presentation, NECs are more likely to be estrogen receptor(ER)/ progesterone receptor (PR) positive and human epidermal growth factor receptor 2 (HER-2) negative, and have ahigher propensity for local recurrence and poorer overall survival(OS). Higher Tclassification, Mclassification, TNM stage, the expression  of Ki67, and the absence of PR expression are prognostically of poorer OS and distant recurrence-free survival(DRFS). Distant metastasis is also adependent prognostic factor. NEC of the breast is adistinct type of neoplasm with higher malignancy. Novel therapies such as the endocrine therapy should be explored and  studies with larger case number and longer follow-up will be needed. Keywords: neuroendocrine, breast cancer, prognosis.

 

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[37]

TÍTULO / TITLE:  - Risk of benign tumours of nervous system, and of malignant neoplasms, in people with neurofibromatosis: population-based record-linkage study.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Br J Cancer. 2013 Jan 15;108(1):193-8. doi: 10.1038/bjc.2012.535. Epub 2012 Dec 20.

            ●● Enlace al texto completo (gratuito o de pago) 1038/bjc.2012.535

AUTORES / AUTHORS:  - Seminog OO; Goldacre MJ

INSTITUCIÓN / INSTITUTION:  - Unit of Health-Care Epidemiology, Department of Public Health, University of Oxford, Old Road Campus, Old Road, Oxford, OX3 7LF, UK.

RESUMEN / SUMMARY:  - Background:The neurofibromatoses (NF) are genetic disorders. Increased risks of some cancers in people with NF are well recognised, but there is no comprehensive enumeration of the risks across the whole range of site-specific cancers. Our aim was to provide this.Methods:A linked data set of hospital admissions and deaths in England was used to compare rates of tumours in an NF cohort with rates in a comparison cohort, with results expressed as rate ratios (RR).Results:The RR for  all cancers combined, in people with both types of NF combined, was 4.3 (95% confidence interval (CI): 4.0-4.6), based on 769 cases of cancer in 8003 people with NF. Considering only people with presumed NF1 (as defined in the main article), the RR for all cancers excluding nervous system malignancies remained elevated (2.7, 95% CI: 2.4-2.9); and risks were significantly high for cancer of  the oesophagus (3.3), stomach (2.8), colon (2.0), liver (3.8), lung (3.0), bone (19.6), thyroid (4.9), malignant melanoma (3.6), non-Hodgkin’s lymphoma (3.3), chronic myeloid leukaemia (6.7), female breast (2.3) and ovary (3.7).Conclusion:Neurofibromatosis was associated with an increased risk of many  individual cancers. The relationships between NF and cancers may hold clues to mechanisms of carcinogenesis more generally.

 

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[38]

TÍTULO / TITLE:  - Vascular E-selectin expression correlates with CD8 lymphocyte infiltration and improved outcome in Merkel cell carcinoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Invest Dermatol. 2013 Jan 25. doi: 10.1038/jid.2013.36.

            ●● Enlace al texto completo (gratuito o de pago) 1038/jid.2013.36

AUTORES / AUTHORS:  - Afanasiev OK; Nagase K; Simonson W; Vandeven N; Blom A; Koelle DM; Clark R; Nghiem P

INSTITUCIÓN / INSTITUTION:  - 1] Department of Medicine/Dermatology, University of Washington, Seattle Washington, USA [2] Department of Pathology, University of Washington, Seattle Washington, USA.

RESUMEN / SUMMARY:  - Merkel cell carcinoma (MCC) is an aggressive, polyomavirus-linked skin cancer. While CD8 lymphocyte infiltration into the tumor is strongly correlated with improved survival, these cells are absent or sparse in most MCCs. We investigated whether specific mechanisms of T-cell migration may be commonly disrupted in MCC  tumors with poor CD8 lymphocyte infiltration. Intratumoral vascular E-selectin, critical for T-cell entry into skin, was downregulated in the majority (52%) of MCCs (n=56), and its loss was associated with poor intratumoral CD8 lymphocyte infiltration (P<0.05; n=45). Importantly, survival was improved in MCC patients whose tumors had higher vascular E-selectin expression (P<0.05). Local nitric oxide (NO) production is one mechanism of E-selectin downregulation and it can be tracked by quantifying nitrotyrosine, a stable biomarker of NO-induced reactive nitrogen species (RNS). Indeed, increasing levels of nitrotyrosine within MCC tumors were associated with low E-selectin expression (P<0.05; n=45) and decreased CD8 lymphocyte infiltration (P<0.05, n=45). These data suggest that one mechanism of immune evasion in MCC may be restriction of T cell entry into the tumor. Existing therapeutic agents that modulate E-selectin expression and/or RNS generation may restore T cell entry and could potentially synergize with other immune-stimulating therapies.Journal of Investigative Dermatology accepted article preview online, 25 January 2013;doi:10.1038/jid.2013.36.

 

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[39]

TÍTULO / TITLE:  - Treatment of Metastatic Neuroendocrine Tumors of the Thymus with Capecitabine and Temozolomide: A Case Series.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Neuroendocrinology. 2013 Jan 3.

            ●● Enlace al texto completo (gratuito o de pago) 1159/000345938

AUTORES / AUTHORS:  - Saranga-Perry V; Morse B; Centeno B; Kvols L; Strosberg J

INSTITUCIÓN / INSTITUTION:  - Department of Oncology, H. Lee Moffitt Cancer Center, Tampa, Fla., USA.

RESUMEN / SUMMARY:  - Background: Metastatic neuroendocrine tumors of the thymus are exceedingly rare with an annual incidence of approximately 0.2 per 1,000,000. They are highly resistant to therapy and there have been no reports of an objective radiographic  response to treatment. Materials and Methods: The authors retrospectively evaluated 3 patients with progressive, metastatic neuroendocrine tumors of the thymus who were treated with a combination of capecitabine and temozolomide. Radiographic scans were evaluated and response assessed using RECIST criteria. Results: One patient experienced a partial radiographic response, another patient experienced a minor response and the third patient experienced stable disease as  the best response to treatment. Conclusion: The combination of capecitabine and temozolomide appears to be active in a rare neuroendocrine malignancy that is generally refractory to systemic therapy. Prospective multicenter trials are needed to validate this strategy.

 

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[40]

TÍTULO / TITLE:  - Deregulation of Wnt/beta-catenin signaling through genetic or epigenetic alterations in human neuroendocrine tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Carcinogenesis. 2013 Jan 25.

            ●● Enlace al texto completo (gratuito o de pago) 1093/carcin/bgt018

AUTORES / AUTHORS:  - Kim JT; Li J; Jang ER; Gulhati P; Rychahou PG; Napier DL; Wang C; Weiss HL; Lee EY; Anthony L; Townsend CM Jr; Liu C; Evers BM

INSTITUCIÓN / INSTITUTION:  - Markey Cancer Center, University of Kentucky, Lexington, Kentucky, USA.

RESUMEN / SUMMARY:  - Carcinoid tumors are rare neuroendocrine tumors (NETs) that are increasing in incidence recently. Mutation and altered expression of Wnt/beta-catenin signaling components have been described in many tumors but have not been well-studied in NETs. Here, we observed accumulation of beta-catenin in the cytoplasm and/or nucleus in 25% of clinical NET tissues. By mutational analysis, the mutations of  beta-catenin (I35S) and APC (E1317Q, T1493T) were identified in NET cells and tissues. Expression of representative cytosolic and extracellular Wnt inhibitors  was absent or markedly decreased inBON, NET cells; treatment with 5-aza-2’-deoxycytidine (5-aza-CdR) increased expression levels of the Wnt inhibitors. Methylation analyses demonstrated that CpG islands of SFRP-1 and Axin-2 were methylated, while the promoters of DKK-1, DKK-3 and WIF-1 were unmethylated. Especially, aberrant methylation of SFRP-1 was observed in most of  clinical NET tissues. In addition, the repressions of these unmethylated genes are associated with histone H3 Lys9 di-methyl modification(H3K9me2) in BON cells. Together, treatment of 5-aza-CdR decreased the protein levels of H3K9me2 and G9a. A novel G9a inhibitor, UNC0638 suppressed BON cell proliferation through inhibition of H3K9me2 and Wnt/beta-catenin pathway. Overexpression of the inhibitory genes, decreased TCF/LEF transcriptional activity and colony formation. In particular, restoration of SFRP-1 and WIF-1 resulted in suppression of anchorage-independent growth and inhibition of tumor growth in mice. Our findings suggest that aberrant Wnt/beta-catenin signaling, through either mutations or epigenetic silencing of Wnt antagonists, contributes to the pathogenesis and growth of NETs and have important clinical implications for the  prognosis and treatment of NETs.

 

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[41]

TÍTULO / TITLE:  - Expression and diagnostic relevance of heat shock protein 90 and signal transducer and activator of transcription 3 in malignant pheochromocytoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Clin Pathol. 2013 Jan 15.

            ●● Enlace al texto completo (gratuito o de pago) 1136/jclinpath-2012-201134

AUTORES / AUTHORS:  - Xu Y; Qi Y; Rui W; Zhu Y; Zhang C; Zhao J; Wei Q; Wu Y; Shen Z; Ning G

INSTITUCIÓN / INSTITUTION:  - Department of Urology, Ruijin Hospital, School of Medicine, Shanghai Jiaotong University, Shanghai, China.

RESUMEN / SUMMARY:  - BACKGROUND: Malignant pheochromocytoma (PCC) is a rare catecholamine producing tumour with a poor prognosis. For many years predicting PCC behaviour has remained a highly difficult task. The aim of this study was to evaluate heat shock protein 90 (HSP90) and signal transducer and activator of transcription 3 (STAT3) as tissue-based markers to predict malignant PCC. METHODS: Ninety-two sporadic PCC patients were enrolled. We compared the expression of HSP90 and STAT3 by immunohistochemistry of benign versus malignant PCCs. In addition, in 17 snap frozen PCC and in 7 healthy adrenal tissues, we investigated the expression  of HSP90 and STAT3 by means of western immunoblot. RESULTS: Positive staining for HSP90 was observed in 22.37% (95% CI 13.00% to 31.74%) of the benign and 66.67% (95% CI 44.89% to 88.45%) of the malignant cases. Similarly, STAT3 staining was seen in 26.32% (95% CI 16.42% to 36.22%) of the benign versus 83.33% (95% CI 66.11% to 100.55%) of the malignant cases. Using HSP90 and STAT3 combined, the positive predictive value of malignancy was signi fi cantly increased to 0.70 (95% CI 0.45 to 0.86). Besides, logistic regression analysis showed that HSP90 (OR=3.667, p=0.039) and STAT3 (OR=9.474, p=0.002) were independently associated with malignant PCC. CONCLUSIONS: This study has con fi rmed that malignant PCC overexpress HSP90 and STAT3, and the combination of HSP90 and STAT3 can be used as helpful diagnostic markers to distinguish malignant from benign PCCs.

 

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[42]

TÍTULO / TITLE:  - Thioredoxin-1 was Required for CREB Activity by Methamphetamine in Rat Pheochromocytoma Cells.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Cell Mol Neurobiol. 2012 Dec 13.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s10571-012-9897-0

AUTORES / AUTHORS:  - Lv T; Wang SD; Bai J

INSTITUCIÓN / INSTITUTION:  - Faculty of Environmental Science and Engineering, Kunming University of Science and Technology, Kunming, 650500, China.

RESUMEN / SUMMARY:  - Methamphetamine (METH) is one of the most commonly abused agents by illicit-drug  users. Thioredoxin-1 (Trx-1) plays important biological roles both in intra- and  extracellular compartments, including in regulation of various intracellular molecules via thiol redox control. In this study, we found that Trx-1 was induced by METH in rat pheochromocytoma PC12 cells. Furthermore, PI3K/Akt pathway was involved in METH-induced increase of Trx-1 expression. An increase in phosphorylated cAMP response element-binding protein (CREB) was also observed after exposure of PC12 cells to METH, which was inhibited by a PI3K inhibitor, LY294002. In addition, the siRNA targeted toTrx-1 reduced the level of phosphorylated CREB by METH, suggesting Trx-1 is necessary for increased activity of CREB by METH. The results obtained in this study showed that Trx-1 might play  a role in the actions of METH.

 

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[43]

TÍTULO / TITLE:  - Exomic Sequencing of Medullary Thyroid Cancer Reveals Dominant and Mutually Exclusive Oncogenic Mutations in RET and RAS.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Clin Endocrinol Metab. 2012 Dec 21.

            ●● Enlace al texto completo (gratuito o de pago) 1210/jc.2012-2703

AUTORES / AUTHORS:  - Agrawal N; Jiao Y; Sausen M; Leary R; Bettegowda C; Roberts NJ; Bhan S; Ho AS; Khan Z; Bishop J; Westra WH; Wood LD; Hruban RH; Tufano RP; Robinson B; Dralle H; Toledo SP; Toledo RA; Morris LG; Ghossein RA; Fagin JA; Chan TA; Velculescu VE; Vogelstein B; Kinzler KW; Papadopoulos N; Nelkin BD; Ball DW

INSTITUCIÓN / INSTITUTION:  - Ludwig Center for Cancer Genetics and Howard Hughes Medical Institutions (N.A., Y.J., M.S., R.L., C.B., N.J.R., V.E.V., B.V., K.W.K., N.P.), Baltimore, Maryland  21287; Department of Otolaryngology-Head and Neck Surgery (N.A., Z.K., R.P.T.), Sidney Kimmel Comprehensive Cancer Center (N.A., S.B., W.H.W., R.P.T., V.E.V., B.V., K.W.K., N.P., B.D.N., D.W.B.), Departments of Neurosurgery (C.B.), Pathology (J.B., W.H.W., L.D.W., R.H.H.), and Medicine (D.W.B.), Johns Hopkins Medical Institutions, Baltimore, Maryland 21287; Human Oncology and Pathogenesis  Program and Departments of Radiation Oncology (T.A.C.), Surgery (A.S.H., T.A.C.), Pathology (R.A.G.), and Endocrinology (J.A.F.) and Head and Neck Service (L.G.T.M.), Memorial Sloan Kettering Cancer Center, New York, New York 10021; University of Sydney (B.R.), New South Wales 2006, Australia; University of Halle-Wittenberg (H.D.), D-06099 Halle, Germany; and Division of Endocrinology (S.P.A.T., R.A.T.), University of Sao Paulo School of Medicine, 05403-900 Sao Paulo, Brazil.

RESUMEN / SUMMARY:  - Context:Medullary thyroid cancer (MTC) is a rare thyroid cancer that can occur sporadically or as part of a hereditary syndrome.Objective:To explore the genetic origin of MTC, we sequenced the protein coding exons of approximately 21,000 genes in 17 sporadic MTCs.Patients and Design:We sequenced the exomes of 17 sporadic MTCs and validated the frequency of all recurrently mutated genes and other genes of interest in an independent cohort of 40 MTCs comprised of both sporadic and hereditary MTC.Results:We discovered 305 high-confidence mutations in the 17 sporadic MTCs in the discovery phase, or approximately 17.9 somatic mutations per tumor. Mutations in RET, HRAS, and KRAS genes were identified as the principal driver mutations in MTC. All of the other additional somatic mutations, including mutations in spliceosome and DNA repair pathways, were not recurrent in additional tumors. Tumors without RET, HRAS, or KRAS mutations appeared to have significantly fewer mutations overall in protein coding exons.Conclusions:Approximately 90% of MTCs had mutually exclusive mutations in RET, HRAS, and KRAS, suggesting that RET and RAS are the predominant driver pathways in MTC. Relatively few mutations overall and no commonly recurrent driver mutations other than RET, HRAS, and KRAS were seen in the MTC exome.

 

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[44]

TÍTULO / TITLE:  - A case of Zollinger-Ellison syndrome diagnosed by duodenal ulcer perforation into the gallbladder.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Gastrointest Endosc. 2013 Jan 25. pii: S0016-5107(12)02996-3. doi: 10.1016/j.gie.2012.11.044.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.gie.2012.11.044

AUTORES / AUTHORS:  - Inuzuka T; Okabe Y; Nishikawa H; Osaki Y

INSTITUCIÓN / INSTITUTION:  - Department of Gastroenterology and Hepatology, Osaka Red Cross Hospital, Osaka City, Osaka, Japan.

 

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[45]

TÍTULO / TITLE:  - Knockdown of Pyruvate Carboxylase or Fatty Acid Synthase Lowers Numerous Lipids and Glucose-Stimulated Insulin Release in Insulinoma Cells.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Arch Biochem Biophys. 2013 Jan 25. pii: S0003-9861(13)00007-6. doi: 10.1016/j.abb.2013.01.002.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.abb.2013.01.002

AUTORES / AUTHORS:  - Macdonald MJ; Hasan NM; Dobrzyn A; Stoker SW; Ntambi JM; Liu X; Sampath H

INSTITUCIÓN / INSTITUTION:  - Childrens Diabetes Center, University of Wisconsin School of Medicine and Public  Health, Madison, WI 53706. Electronic address: mjmacdon@wisc.edu.

RESUMEN / SUMMARY:  - We previously showed that knockdown of the anaplerotic enzyme pyruvate carboxylase in the INS-1 832/13 insulinoma cell line inhibited glucose-stimulated insulin release and glucose carbon incorporation into lipids. We now show that knockdown of fatty acid synthase (FAS) mRNA and protein also inhibits glucose-stimulated insulin release in this cell line. Levels of numerous phospholipids, cholesterol esters, diacylglycerol, triglycerides and individual fatty acids with C(14)-C(24) side chains were acutely lowered about 20% in glucose-stimulated pyruvate carboxylase knockdown cells over a time course that coincides with insulin secretion. In FAS knockdown cells glucose carbon incorporation into lipids and the levels of the subclasses of phospholipids and cholesterol ester species were lower by 20-30% without inhibition of glucose oxidation. These studies suggest that rapid lipid modification is essential for normal glucose-stimulated insulin secretion.

 

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[46]

TÍTULO / TITLE:  - Pheochromocytoma Crisis Is Not a Surgical Emergency.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Clin Endocrinol Metab. 2013 Jan 2.

            ●● Enlace al texto completo (gratuito o de pago) 1210/jc.2012-3020

AUTORES / AUTHORS:  - Scholten A; Cisco RM; Vriens MR; Cohen JK; Mitmaker EJ; Liu C; Tyrrell JB; Shen WT; Duh QY

INSTITUCIÓN / INSTITUTION:  - Section of Endocrine Surgery (A.S., R.M.C., J.K.C., E.J.M., W.T.S., Q.-Y.D.), University of California, San Francisco, California 94143; Department of Surgical Oncology and Endocrine Surgery (A.S., M.R.V.), University Medical Center Utrecht  3584 CG, The Netherlands; Division of Endocrinology and Metabolism (C.L., J.B.T.), Department of Medicine, University of California, San Francisco, California 94143.

RESUMEN / SUMMARY:  - Context:Pheochromocytoma crisis is a feared and potentially lethal complication of pheochromocytoma.Objective:We sought to determine the best treatment strategy  for pheochromocytoma crisis patients and hypothesized that emergency resection is not indicated.Design:Retrospective cohort study (1993-2011); literature review (1944-2011).Setting:Tertiary referral center.Patients:There were 137 pheochromocytoma patients from our center and 97 pheochromocytoma crisis patients who underwent adrenalectomy from the literature.Intervention:Medical management of pheochromocytoma crisis; adrenalectomy.Main Outcome Measure(s):Perioperative complications, conversion, and mortality.Results:In our database, 25 patients (18%) presented with crisis. After medical stabilization and alpha-blockade, 15 patients were discharged and readmitted for elective surgery and 10 patients were operated on urgently during the same hospitalization. None underwent emergency surgery. Postoperatively, patients who underwent elective surgery had shorter hospital stays (1.7 vs 5.7 d, P = 0.001) and fewer postoperative complications (1 of 15 [7%] vs 5 of 10 [50%], P = 0.045) and were less often admitted to the intensive care unit (1 of 15 [7%] vs 5 of 10 [50%], P = 0.045) in comparison with urgently operated patients. There was no mortality. Review of the literature (n = 97) showed that crisis patients who underwent elective or urgent surgery vs emergency surgery had less intraoperative (13 of 31 [42%] vs 20 of 25 [80%], P <  0.001) and postoperative complications (15 of 45 [33%] vs 15 of 21 [71%], P = 0.047) and a lower mortality (0 of 64 vs 6 of 33 [18%], P = 0.002).Conclusions:Management of patients presenting with pheochromocytoma crisis should include initial stabilization of the acute crisis followed by sufficient alpha-blockade before surgery. Emergency resection of pheochromocytoma is associated with high surgical morbidity and mortality.

 

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[47]

TÍTULO / TITLE:  - Merkel cell polyomavirus infection in both components of a combined Merkel cell carcinoma and basal cell carcinoma with ductal differentiation; each component had a similar but different novel Merkel cell polyomavirus large T antigen truncating mutation.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Hum Pathol. 2012 Dec 20. pii: S0046-8177(12)00325-5. doi: 10.1016/j.humpath.2012.08.022.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.humpath.2012.08.022

AUTORES / AUTHORS:  - Iwasaki T; Kodama H; Matsushita M; Kuroda N; Yamasaki Y; Murakami I; Yamamoto O; Hayashi K

INSTITUCIÓN / INSTITUTION:  - Division of Molecular Pathology, Department of Pathology, Tottori University Faculty of Medicine, Nishi-cho 86, Yonago, Tottori, 683-8503, Japan. Electronic address: iwasakit-path@umin.ac.jp.

RESUMEN / SUMMARY:  - Merkel cell polyomavirus infects up to 80% of patients with Merkel cell carcinoma. Combined Merkel cell carcinoma and cutaneous tumors occur occasionally. Previous reports have suggested that Merkel cell polyomavirus is absent from combined Merkel cell carcinoma and squamous cell carcinomas. This is  the first report that Merkel cell polyomavirus infected in both lesions of a combined Merkel cell carcinoma and basal cell carcinoma. A 92-year-old Japanese man presented with a right thigh small subcutaneous mass. Histologic examination  revealed a combined tumor with Merkel cell carcinoma and basal cell carcinoma with ductal differentiation. Both tumors and intermingled Merkel cells in basal cell carcinoma expressed Merkel cell polyomavirus large T antigen, and 17 and 240 copies of Merkel cell polyomavirus/cell were detected in the microdissected Merkel cell carcinoma and basal cell carcinoma specimens, respectively. Mutation  analysis of Merkel cell polyomavirus large T antigen revealed a novel truncating  mutation in Merkel cell carcinoma and a similar but different mutation in the basal cell carcinoma. These results suggest that each was infected by a different Merkel cell polyomavirus subclone derived from a single Merkel cell polyomavirus.

 

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[48]

TÍTULO / TITLE:  - Gastroenteropancreatic neuroendocrine tumors: role of imaging in diagnosis and management.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Radiology. 2013 Jan;266(1):38-61. doi: 10.1148/radiol.12112512.

            ●● Enlace al texto completo (gratuito o de pago) 1148/radiol.12112512

AUTORES / AUTHORS:  - Sahani DV; Bonaffini PA; Fernandez-Del Castillo C; Blake MA

INSTITUCIÓN / INSTITUTION:  - Department of Radiology, Division of Abdominal Imaging and Interventional Radiology, and Department of Surgery, Massachusetts General Hospital, Harvard Medical School, 55 Fruit St, White 270, Boston, MA 02114.

RESUMEN / SUMMARY:  - Gastroenteropancreatic neuroendocrine tumors (GEP-NETs) are a heterogeneous group of neoplasms that arise from cells of the diffuse neuroendocrine system and are characterized by a wide spectrum of clinical manifestations. All NETs are potentially malignant but differ in their biologic characteristics and the probability of metastatic disease. The pathologic classification of these tumors  relies on their proliferation and differentiation. In the past decades, several nomenclatures have been proposed to stratify neuroendocrine tumors, but the World Health Organization classification is the one that is most widely accepted and used. The diagnosis of neuroendocrine tumor relies on clinical manifestation, laboratory parameters, imaging features, and tissue biomarkers in a biopsy specimen. With improved understanding of the natural history and lesion biology,  management of GEP-NETs has also evolved. Although surgery remains the only potentially curative therapy for patients with primary GEP-NETs, other available  treatments include chemotherapy, interferon, somatostatin analogs, and targeted therapies. Recent improvements in both morphologic and functional imaging methods have contributed immensely to patient care. Morphologic imaging with contrast agent-enhanced multidetector computed tomography and magnetic resonance imaging is most widely used for initial evaluation and staging of disease in these patients, whereas functional imaging techniques are useful both for detection and prognostic evaluation and can change treatment planning. ©RSNA, 2013.

 

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[49]

TÍTULO / TITLE:  - Malignant pheochromocytoma and paraganglioma: A population level analysis of long-term survival over two decades.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Surg Oncol. 2012 Dec 11. doi: 10.1002/jso.23297.

            ●● Enlace al texto completo (gratuito o de pago) 1002/jso.23297

AUTORES / AUTHORS:  - Goffredo P; Sosa JA; Roman SA

INSTITUCIÓN / INSTITUTION:  - Department of Surgery, Milano-Bicocca University, Monza, Italy.

RESUMEN / SUMMARY:  - BACKGROUND AND OBJECTIVES: Pheochromocytoma (PHEO) and paraganglioma (PGL) are rare tumors. Aims of this study were to describe and to compare demographic, clinical, pathologic, and survival characteristics of malignant PHEO and PGL. METHODS: Patients were identified in SEER, 1988-2009. Analyses included chi-square, ANOVA, Kaplan-Meier, and Cox proportional hazard regression. RESULTS: Gender distribution and mean age were similar for PHEO and PGL. Surgery was performed in 74.3% of PHEO and 78.9% of PGL; external beam radiation was administered in 8.0% of PHEO and 28.1% of PGL (P < 0.001). Compared to PGL, PHEO  were larger (mean size 7.7 vs. 4.5 cm PGL, P = 0.001) and more were SEER-staged as localized (17.3% vs. 49.6%, respectively, P < 0.001). PGLs were more often located in the trunk than in the head/neck (53.8% vs. 38.0%, P < 0.001). PHEO had lower overall and disease-specific survival than PGL (54.0% and 73.5% vs. 73.3% and 80.5% for PGL, respectively, P < 0.001 and P = 0.118). Independent factors associated with mortality for PHEO included not undergoing surgery and metastases at diagnosis; for PGL, these were age 61-75 years, size >/=5 cm, and presenting with metastases. CONCLUSIONS: Malignant PHEO has a more aggressive course than malignant PGL; long-term survival has not improved over the last two decades. Multi-institutional efforts should be pursued to seek novel treatments. J. Surg.  Oncol © 2012 Wiley Periodicals, Inc.

 

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[50]

TÍTULO / TITLE:  - NF1 deficiency causes Bcl-xL upregulation in Schwann cells derived from neurofibromatosis type 1-associated malignant peripheral nerve sheath tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Int J Oncol. 2013 Feb;42(2):657-66. doi: 10.3892/ijo.2012.1751. Epub 2012 Dec 24.

            ●● Enlace al texto completo (gratuito o de pago) 3892/ijo.2012.1751

AUTORES / AUTHORS:  - Park HJ; Lee SJ; Sohn YB; Jin HS; Han JH; Kim YB; Yim H; Jeong SY

INSTITUCIÓN / INSTITUTION:  - Department of Medical Genetics, Ajou University School of Medicine, Suwon 443-721, Republic of Korea.

RESUMEN / SUMMARY:  - Since the bi-allelic inactivation of both neurofibromin 1 (NF1) gene alleles (NF1-/-) in Schwann cells (SCs) is common in both benign plexiform neurofibromas  (PNs) and malignant peripheral nerve sheath tumors (MPNSTs) in patients with neurofibromatosis type 1 (NF1), other genetic alterations in SCs may be required  for tumor progression of PNs to MPNSTs. We found that the anti-apoptotic Bcl-xL protein is upregulated in MPNST tissues compared to PN tissues from patients with NF1 by immunohistological staining. In addition, we investigated whether Bcl-xL is upregulated in SCs derived from MPNSTs and found a significantly higher Bcl-xL expression level in sNF96.2 MPNST-derived SCs compared to normal human SCs (HSCs). We also discovered that the increased Bcl-xL expression caused an increase in drug resistance to doxorubicin in MPNST-derived SCs. Manipulation of  NF1 gene expression levels by treatment with small interfering RNA (siRNA) and overexpression of the neurofibromin GAP-related domain (NF1-GRD) demonstrated that upregulated Bcl-xL expression in MPNST-derived SCs was caused by NF1 deficiency. Treatment with the Erk1/2 inhibitor, PD98059, resulted in a slight increase in Bcl-xL levels in neurofibromin-depleted normal HSCs, indicating that  Bcl-xL upregulation in MPNST-derived SCs is mediated by activated Erk1/2, which is a Ras downstream protein regulated by neurofibromin. As the reduction of Bcl-xL expression restored sensitivity to doxorubicin-induced apoptosis in sNF96.2 cells, we examined the effect of the small molecule Bcl-xL inhibitor ABT-737 on sNF96.2 cells. A very low dose of ABT-737 combined with doxorubicin synergistically enhanced sensitivity to doxorubicin-induced apoptosis in sNF96.2  cells, suggesting that ABT-737 and doxorubicin may be a good combination to effectively treat NF1-associated MPNSTs with minimal side-effects. Collectively,  our results suggest that upregulation of Bcl-xL in MPNST-derived SCs may be caused by the NF1 deficiency-mediated elevation in Ras/MAPK signaling and may provide a new potential chemotherapeutic target in patients with NF1 and MPNSTs.

 

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[51]

TÍTULO / TITLE:  - Neuroendocrine small cell carcinoma of the breast—a case report.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Coll Antropol. 2012 Sep;36(3):1053-5.

AUTORES / AUTHORS:  - Zadro Z; Fuduric J; Frketic I; Stifter S; Bujas T; Zadro AS; Veir Z; Doko I

INSTITUCIÓN / INSTITUTION:  - Karlovac General Hospital, Department of Surgery, Karlovac, Croatia. zvonko.zadro@zg.htnet.hr

RESUMEN / SUMMARY:  - Neuroendocrine tumors are very rare tumors that occur most commonly in the gastrointestinal tract. The occurrence of neuroendocrine tumors outside gastrointestinal tract is very rare but not unknown. Thus, neuroendocrine tumors  and their primary seat can be found in the bronchi and lungs, as well as in the testicles, ovaries, prostate, etc. The occurrence of neuroendocrine tumors as a primary seat in the breast is extremely rare phenomenon that is described in literature. We present the case of 55-year old female in where routine mammographic examination found suspicious lesions that we recommended for further processing. The patient made a breast ultrasound examination in which tumor formation was found in size 27 x 19 mm and cytological puncture found breast adenocarcinoma. Further pathohystologic and immunohistochemical analysis set the  diagnosis of neuroendocrine carcinoma, small cell type, second grade. Tumor formation by ultrasound initially sized 27 x 19 mm and pathohistologic diagnosis  showed tumor size 26 x 20 x 20 mm. The axillary lymph node biopsy did not found distant metastases in lymph nodes as well as gatherings in other organs. Neuroendocrine small cell carcinomas are exceedingly rare phenomena in the literature. By the year 2009 in the USA there were described only 50 cases of this extremely rare tumor of the breast.

 

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[52]

TÍTULO / TITLE:  - Surgical Treatment for Limited-Stage Primary Small Cell Cancer of the Esophagus.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Ann Thorac Surg. 2013 Jan 17. pii: S0003-4975(12)02577-5. doi: 10.1016/j.athoracsur.2012.11.014.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.athoracsur.2012.11.014

AUTORES / AUTHORS:  - Situ D; Lin Y; Long H; Zhang L; Lin P; Zheng Y; Jiang L; Tan Z; Meng Y; Ma G

INSTITUCIÓN / INSTITUTION:  - State Key Laboratory of Oncology in South China, Guangzhou, P. R. China; Department of Thoracic Surgery, Cancer Center, Sun Yat-sen University, Guangzhou, P. R. China.

RESUMEN / SUMMARY:  - BACKGROUND: Primary small cell cancer of the esophagus (PSCCE) is a rare, aggressive, and highly metastatic disease. Surgical intervention, radiotherapy, and chemotherapy have been used alone or in combination to improve survival. This retrospective study tried to evaluate the significance of surgical procedures for the treatment of limited-stage PSCCE. METHODS: We retrospectively evaluated 44 patients with limited-stage PSCCE who received esophagectomy with lymphadenectomy in our center between 1994 and 2011. The clinical and pathologic characteristics, median survival time (MST), overall survival (OS), and relevant prognostic factors were analyzed. RESULTS: The MST in our cohort was 18.0 months (95% confidence interval [CI], 9.6-26.4 months), and the 6-, 12-, 24-, 36-, and 60-month OS rates were 73%, 58%, 39%, 30%, and 18%, respectively. The MST of patients with positive lymph nodes was significantly shorter than that of those with negative lymph nodes (14 months versus 47 months; p = 0.031). Survival analysis confirmed that regional lymph node involvement (relative risk [RR], 5.287; 95% CI, 1.036-26.978; p = 0.045) was an independent prognostic factor. CONCLUSIONS: Although the standard treatment protocol for PSCCE has not been established, the results of our study indicated that radical esophagectomy with extended lymphadenectomy should be considered as the primary treatment for patients with limited-stage PSCCE, particularly for those without regional lymph  node involvement.

 

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[53]

TÍTULO / TITLE:  - Immunohistochemical expression and prognostic significance of HIF-1alpha and VEGF-C in neuroendocrine breast cancer.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Anticancer Res. 2012 Dec;32(12):5227-32.

AUTORES / AUTHORS:  - Marton I; Knezevic F; Ramic S; Milosevic M; Tomas D

INSTITUCIÓN / INSTITUTION:  - Clinic for Gynaecology and Obstetrics, Sveti Duh University Hospital, Sveti Duh 64, 10 000 Zagreb, Croatia. ingridmarton@gmail.com

RESUMEN / SUMMARY:  - AIM: To determine the predictive value of HIF-1alpha and VEGF-C in primary neuroendocrine breast cancers (NEBC) and their correlation with other clinico-pathological characteristics of NEBC. MATERIALS AND METHODS: HIF-1alpha and VEGF-C were determined immunohistochemically in tissue samples from 31 cases  of NEBC. RESULTS: The HIF-1alpha expression in NEBC was predominantly negative, with only 5 (16.1%) cases showing strong reaction to HIF-1alpha. Eighteen (58.0%) NEBC cases showed moderate-to-strong VEGF-C expression. VEGF-C expression negatively correlated with progesterone receptor positivity (p=0.014) and duration of follow-up (p=0.021). A multivariate Cox proportional hazard regression analysis showed that HIF-1alpha (p=0.019) was a significant predictor  of disease-free survival, whereas VEGF-C (p=0.099) showed no such association. CONCLUSION: HIF-1alpha overexpression indicated unfavourable prognosis and could  serve as an additional prognostic factor in NEBC. Moreover, patients with NEBC exhibiting moderate or strong VEGF-C expression could be candidates for a specific VEGF-C antibody therapy.

 

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[54]

TÍTULO / TITLE:  - Pheochromocytoma: The Range of Appearances on Ultrasound, CT, MRI, and Functional Imaging.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - AJR Am J Roentgenol. 2013 Feb;200(2):370-8. doi: 10.2214/AJR.12.9126.

            ●● Enlace al texto completo (gratuito o de pago) 2214/AJR.12.9126

AUTORES / AUTHORS:  - Leung K; Stamm M; Raja A; Low G

INSTITUCIÓN / INSTITUTION:  - Department of Radiology & Diagnostic Imaging, University of Alberta Hospital, 2A2.41 Walter Mackenzie Centre, 8440-112 St, Edmonton, AB, T6G 2B7, Canada.

RESUMEN / SUMMARY:  - OBJECTIVE: Pheochromocytomas are relatively rare neuroendocrine tumors of the adrenal medulla. Their variable clinical presentation and biologic behavior often make accurate diagnosis challenging. A variable spectrum of imaging appearances-some of which may also mimic other diseases-has been recognized. This article reviews the epidemiology; associations; and clinical, biochemical, pathologic, and multimodality imaging features of pheochromocytomas including diagnostic pearls and pitfalls. CONCLUSION: Pheochromocytomas are often considered the great mimicker of other adrenal tumors. Because of their varied clinical, imaging, and pathologic appearances, accurate diagnosis can be challenging. The various imaging appearances on ultrasound, CT, MRI, and functional imaging can be complementary and have features that are useful for differentiating pheochromocytoma from other lesions of the adrenal.

 

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[55]

TÍTULO / TITLE:  - Islet-1 Is a Sensitive But Not Entirely Specific Marker for Pancreatic Neuroendocrine Neoplasms and Their Metastases.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Am J Surg Pathol. 2013 Jan 22.

            ●● Enlace al texto completo (gratuito o de pago) 1097/PAS.0b013e31826f042c

AUTORES / AUTHORS:  - Graham RP; Shrestha B; Caron BL; Smyrk TC; Grogg KL; Lloyd RV; Zhang L

INSTITUCIÓN / INSTITUTION:  - Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN.

RESUMEN / SUMMARY:  - Islet-1 (Isl1) is a transcription factor involved in the embryogenesis of islets  of Langerhans. Immunohistochemically, Isl1 is a sensitive lineage-specific marker for pancreatic neuroendocrine neoplasms (NENs) and their metastases. Its specificity has not been documented, nor have large numbers of NENs from other parts of the gut or other organs been studied. We examined Isl1 expression in 203 primary NENs (gastroenteropancreatic, lung, breast, and ovarian neoplasms) and 40 hepatic NEN metastases (enteropancreatic and lung neoplasms) from known primaries. The correlation between Isl1 and CDX2 expression was studied using a tissue microarray containing 46 pancreatic NENs. Immunostaining for Isl1 and CDX2 was also performed in selected NENs from other sites. Isl1 was positive in 90% of pancreatic, 89% of duodenal, 100% of rectal, 38% of colonic, 14% of appendiceal,  and 6% of ileal primaries. Isl1 was negative in all other NENs. Among metastatic  neoplasms, 76% of pancreatic and 2 of 2 rectal NEN metastases were Isl1 positive, whereas all other tested metastases were negative. The overall sensitivity and specificity of Isl1 in identifying primary pancreatic NENs was 88% and 80%, respectively. Thirty-six of 46 pancreatic NENs in the tissue microarray were Isl1 positive; 4 were Isl1 negative but CDX2 positive. Our findings confirm that Isl1  is a sensitive marker of pancreatic origin in cases of metastatic NEN. However, Isl1 does not distinguish pancreatic NEN from duodenal and colorectal NEN, even when used in association with CDX2.

 

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[56]

TÍTULO / TITLE:  - Staging and treatment response evaluation in a metastatic neuroendocrine tumor of the pancreas with G2 grading: insights from multimodality diagnostic approach by  F-18-FDG and Ga-68-DOTANOC PET/CT.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endocrine. 2012 Dec 18.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s12020-012-9858-x

AUTORES / AUTHORS:  - Treglia G; Plastino F; Campitiello M

INSTITUCIÓN / INSTITUTION:  - Institute of Nuclear Medicine, Catholic University of the Sacred Heart, Largo A.  Gemelli 8, 00168, Rome, Italy, giorgiomednuc@libero.it.

 

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[57]

TÍTULO / TITLE:  - Renal Medullary Carcinoma: Molecular, Immunohistochemistry, and Morphologic Correlation.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Am J Surg Pathol. 2013 Jan 22.

            ●● Enlace al texto completo (gratuito o de pago) 1097/PAS.0b013e3182770406

AUTORES / AUTHORS:  - Liu Q; Galli S; Srinivasan R; Linehan WM; Tsokos M; Merino MJ

INSTITUCIÓN / INSTITUTION:  - Translational Surgical Pathology and Urologic Oncology sections, National Cancer  Institute, Bethesda, MD.

RESUMEN / SUMMARY:  - Renal medullary carcinoma, a highly aggressive tumor mainly occurring in patients with sickle cell hemoglobinopathy, is characterized by advanced stage at the time of presentation and poor response to treatment. Currently, the pathogenesis of this tumor is not well understood. In this study, the clinicopathologic features  and molecular changes of 15 renal medullary carcinoma cases were evaluated. These cases demonstrated male predominance (M:F=2:1) with a median age of 26 years. The tumors occurred predominantly in the right kidney with an average size of 5.9 cm. Immunohistochemistry analysis showed that the neoplastic cells were positive for  CEA (7/8), AE1/3 (8/8), CAM5.2 (7/7), CK7 (5/5), CK20 (4/6), and vimentin (6/6).  Absence of SMARCB1 protein expression in tumor cells was demonstrated in all of the 7 cases analyzed. By polymerase chain reaction-based microsatellite analysis, loss of heterozygosity of SMARCB1 was identified in 9 of 10 cases. These data suggest that inactivation of SMARCB1 may play a role in the pathogenesis of renal medullary carcinoma.

 

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[58]

TÍTULO / TITLE:  - Immunohistochemical markers of the hypoxic response can identify malignancy in phaeochromocytomas and paragangliomas and optimize the detection of tumours with  VHL germline mutations.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Br J Cancer. 2012 Dec 20. doi: 10.1038/bjc.2012.538.

            ●● Enlace al texto completo (gratuito o de pago) 1038/bjc.2012.538

AUTORES / AUTHORS:  - Pinato DJ; Ramachandran R; Toussi ST; Vergine M; Ngo N; Sharma R; Lloyd T; Meeran K; Palazzo F; Martin N; Khoo B; Dina R; Tan TM

INSTITUCIÓN / INSTITUTION:  - 1] Department of Investigative Medicine, Imperial College London, Hammersmith Hospital Campus, Du Cane Road, London W12 0NN, UK [2] Division of Internal Medicine, Department of Clinical and Experimental Medicine, Universita degli Studi del Piemonte Orientale, Via Solaroli 17, Novara, Italy.

RESUMEN / SUMMARY:  - Background:There are no reliable markers of malignancy in phaeochromocytomas (PCC) and paragangliomas (PGL). We investigated the relevance of the mammalian target of rapamycin (mTOR)/AKT and hypoxic pathways as novel immunohistochemical  markers of malignancy.Methods:Tissue microarray blocks were constructed with a total of 100 tumours (10 metastatic) and 20 normal adrenomedullary samples. Sections were immunostained for hypoxia-inducible factor 1alpha (Hif-1alpha), vascular endothelial growth factor A (VEGF-A), mTOR, carbonic anhydrase IX (CaIX) and AKT. The predictive performance of these markers was studied using univariate, multivariate and receiver operating characteristic analyses.Results:In all, 100 consecutive patients, 64% PCC, 29% familial with a median tumour size of 4.7 cm (range 1-14) were included. Univariate analyses showed Hif-1alpha overexpression, tumour necrosis, size >5 cm, capsular and vascular invasion to be predictors of metastasis. In multivariate analysis, Hif-1alpha, necrosis and vascular invasion remained as independent predictors of  metastasis. Hif-1alpha was the most discriminatory biomarker for the presence of  metastatic diffusion. Strong membranous CaIX expression was seen in von Hippel-Lindau (VHL) PCC as opposed to other subtypes.Conclusion:Lack of vascular  invasion, tumour necrosis and low Hif-1alpha expression identify tumours with lower risk of malignancy. We propose membranous CaIX expression as a potential marker for VHL disease in patients presenting with PCC.British Journal of Cancer  advance online publication, 20 December 2012; doi:10.1038/bjc.2012.538 www.bjcancer.com.

 

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[59]

TÍTULO / TITLE:  - Incidence and survival of neuroendocrine tumours in the Netherlands according to  histological grade: Experience of two decades of cancer registry.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Eur J Cancer. 2013 Jan 23. pii: S0959-8049(12)01031-3. doi: 10.1016/j.ejca.2012.12.022.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.ejca.2012.12.022

AUTORES / AUTHORS:  - Korse CM; Taal BG; van Velthuysen ML; Visser O

INSTITUCIÓN / INSTITUTION:  - Department of Clinical Chemistry, The Netherlands Cancer Institute - Antoni van Leeuwenhoek Hospital, The Netherlands. Electronic address: t.korse@nki.nl.

RESUMEN / SUMMARY:  - PURPOSE: Epidemiological studies on neuroendocrine tumours (NETs) generally show  a major increase in incidence. To investigate this increase, epidemiological data from the Netherlands were evaluated according to histological grade. METHODS: All 47,800 patients with NET (diagnosed 1990-2010) from the population-based Netherlands Cancer Registry were stratified according to the latest World Health  Organization (WHO) classification for the digestive system: well-differentiated NET grade 1 and 2 (G1NET and G2NET), and poorly differentiated (grade 3) neuroendocrine carcinoma, subdivided into large cell (G3-LCNEC) and small cell (G3-SCNEC). RESULTS: The age-standardised incidence rate (excluding G3-SCNEC) increased from 2.1/100,000 in 1990 to 4.9/100,000 in 2010. The incidence of G1NET increased from 2.0 to 3.0; there was a large increase in G2NET from 0.01 in 1990  to 0.2 in 2010, and of the G3-LCNEC from 0.01 to 1.8, respectively. In G3-SCNEC incidence in men decreased from 21.3 to 10.1, whereas in women it increased from  4.5 to 7.7. The 5-year survival improved in G1NET, particularly for metastatic disease, from 30% in 1990-2000 to 47% in 2001-2010. CONCLUSION: The increasing incidence of NET (without G3-SCNEC) was mainly due to the increase of G3-LCNEC. This increase is related to improved diagnostic procedures and to shifting in pathology from other entities (such as undifferentiated carcinoma) to NET. Improved survival was seen in all sites and stages, especially in patients with metastatic G1NET. Because of its influence on incidence and survival, we recommend to present epidemiological studies on NET according to histological classification.

 

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[60]

TÍTULO / TITLE:  - TrkB/BDNF signaling pathway is a potential therapeutic target for pulmonary large cell neuroendocrine carcinoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Lung Cancer. 2013 Jan 8. pii: S0169-5002(12)00649-6. doi: 10.1016/j.lungcan.2012.12.004.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.lungcan.2012.12.004

AUTORES / AUTHORS:  - Odate S; Nakamura K; Onishi H; Kojima M; Uchiyama A; Nakano K; Kato M; Tanaka M; Katano M

INSTITUCIÓN / INSTITUTION:  - Department of Cancer Therapy and Research, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.

RESUMEN / SUMMARY:  - Tropomyosin-related kinase B (TrkB) plays an important role in tumor progression  in various kinds of cancers; however, little is known about biological significance of TrkB in human lung cancer, especially large cell neuroendocrine carcinoma (LCNEC). We hereby investigated the expressions of TrkB and its ligand  brain-derived neurotrophic factor (BDNF) in clinical specimens and their influences on phenotypes of invasiveness and tumorigenicity for LCNEC. The expressions of TrkB and BDNF analyzed by immunohistochemistry for patients samples with lung cancer (n=104) were significantly higher in neuroendocrine tumor (NET) compared with non-NET. In particular, LCNEC, a subtype of NET, exhibited significantly higher TrkB and BDNF expressions than another NET type: small cell lung cancer (SCLC), and a significant correlation between TrkB and BDNF expressions was noted in LCNEC but not in SCLC. In vitro assay, exogenous BDNF addition enhanced the invasion into matrigels of LCNEC cells, whereas inhibition of TrkB or BDNF suppressed matrix metalloproteinase-2 and -9 activities and the invasiveness. Exogenous BDNF also increased anchor-independent colony formation on soft agar gels for LCNEC, while inhibition of TrkB or BDNF suppressed the anchorage-independency. In vivo experiments, implanted LCNEC cells pretreated with TrkB-siRNA developed no subcutaneous tumor in all six nude mice,  although those with control-siRNA formed tumors in four of six nude mice. In conclusion, BDNF/TrkB signal is involved in malignant progression of invasiveness and tumorigenicity for LCNEC, and may be a potential target for LCNEC without standard therapy.

 

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[61]

TÍTULO / TITLE:  - International Practice Patterns and Resource Utilization in the Treatment of Neuroendocrine Tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Pancreas. 2013 Jan 25.

            ●● Enlace al texto completo (gratuito o de pago) 1097/MPA.0b013e31826707cc

AUTORES / AUTHORS:  - Casciano R; Wang X; Stern L; Parikh R; Chulikavit M; Willet J; Liu Z; Strosberg J; Cadiot G; Riechelmann R

INSTITUCIÓN / INSTITUTION:  - From the *LA-SER Analytica, New York, NY; daggerNovartis Oncology, Florham Park,  NJ; double daggerH. Lee Moffitt Cancer Center & Research Institute, Experimental  Therapeutics Gastrointestinal Tumor, Tampa, FL; section signService d’Hepato-Gastroenterologie et de Cancerologie Digestive Hopital Robert Debre, Reims Cedex, France; and parallelInstituto do Cancer do Estado de Sao Paulo, Clinical Research, Sao Paulo, Brazil.

RESUMEN / SUMMARY:  - OBJECTIVES: This study compared resource use and practice patterns in patients with advanced neuroendocrine tumors (NETs) on disease progression, across countries, and by tumor type. METHODS: Physicians in the United States, United Kingdom, Germany, France, Brazil, and Italy completed data extraction forms to extract chart data of patients with NET relating to health care resource utilization and treatment practice. Data were assessed in a cross-sectional manner, by country, and by NET subtype. Univariate and multivariate analyses were performed to compare categories of resource use by disease progression status. RESULTS: A total of 197 physicians provided data on 394 patients. Overall resource utilization was high across tumor types, countries, and progression. Nearly half of all patients received chemotherapy (49%); moreover, high rates of  hospitalization (65%), surgery (47%), and use of somatostatin analog (77%) were observed, with lower rates of peptide receptor radionuclide therapy (10%) and targeted therapies (6%). These patterns were consistent across gastrointestinal tract/lung NET and pancreatic NET. However, a certain variation in resource utilization was observed across countries. Disease progression was associated with increasing utilization of chemotherapy, hospitalization, and targeted therapy. CONCLUSIONS: Advanced NET is associated with significant resource use across subtypes and countries, and resource utilization is likely to increase on  disease progression. There remains an unmet need for therapeutic options after disease progression.

 

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[62]

TÍTULO / TITLE:  - Current and Future Therapeutic Approaches for Metastatic Pheochromocytoma and Paraganglioma: Focus on SDHB Tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Horm Metab Res. 2013 Jan 15.

            ●● Enlace al texto completo (gratuito o de pago) 1055/s-0032-1331211

AUTORES / AUTHORS:  - Matro J; Giubellino A; Pacak K

INSTITUCIÓN / INSTITUTION:  - Program in Reproductive and Adult Endocrinology, Eunice Kennedy Shriver National  Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA.

RESUMEN / SUMMARY:  - As a result of intense genetic studies of families with specific mutations, the road to better therapeutic intervention for pheochromocytoma (PHEOs) and parangangliomas (PGLs) has more recently become populated with several promising  molecular targets. Consequently a change in paradigm from a previous view on nonspecific therapy has shifted towards more selective molecular targeted therapies. In particular, malignant PHEOs/PGLs, more specifically the tumors that result from mutations in succinate dehydrogenase subunit B (SDHB), are a clear concern, and novel therapies should be developed to address this problem. Here we summarize current and future therapeutic approaches.

 

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[63]

TÍTULO / TITLE:  - Production and quality control of [90Y]DOTATOC for treatment of metastatic neuroendocrine tumors: results of 85 syntheses.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Nucl Med Commun. 2013 Mar;34(3):265-70. doi: 10.1097/MNM.0b013e32835ce596.

            ●● Enlace al texto completo (gratuito o de pago) 1097/MNM.0b013e32835ce596

AUTORES / AUTHORS:  - Biasiotto G; Bertagna F; Zanella I; Biasiotto U; Savelli G; Caimi L; Bettinsoli G; Giubbini R; Chinol M

INSTITUCIÓN / INSTITUTION:  - aSection of Biochemistry bSection of Nuclear Medicine, Faculty of Medicine, University of Brescia cLaboratory of Biotechnology dDepartment of Nuclear Medicine, Spedali Civili, Brescia eItalian Army, Ministry of Defence, Rome fDepartment of Nuclear Medicine, A. O. Carlo Poma Hospital, Mantova gDivision of  Nuclear Medicine, European Institute of Oncology, Milano, Italy.

RESUMEN / SUMMARY:  - The aim of this paper was to describe the optimized labeling protocol and quality control measures used in the production of [Y]DOTATOC, starting from three different radioactivity levels to treat one, two, or three patients per therapeutic session. We investigated three different starting radioactivity levels. For the low radioactivity preparation we used 5138+/-280 MBq of [Y] isotope, for the medium radioactivity preparation we used 8893+/-900 MBq, and for the high radioactivity preparation we used 11250+/-715 MBq. The radiochemical purity levels for the low radioactivity preparation, medium radioactivity preparation, and high radioactivity preparation were 99.95+/-0.09, 99.84+/-0.34,  and 99.84+/-0.53%, respectively, and the radiochemical yields of the labeling procedures were 77.52+/-1.28, 75.53+/-3.72, and 78.00+/-3.20%, respectively. Media fill validation of the process was performed, and the parameters of pH, bacterial endotoxins, sterility, and osmolality were tested at process control. All radiopharmaceutical preparations satisfied the predetermined specifications fixed in our protocol regardless of the starting radioactivity level. The validation of the method guaranteed the safety and quality of the final products, contributing to providing the basis for constructing an informative and successful clinical trial.

 

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[64]

TÍTULO / TITLE:  - Effect of Carnosine and Related Compounds on Proliferation of Cultured Rat Pheochromocytoma PC-12 Cells.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Bull Exp Biol Med. 2012 Oct;154(1):136-40.

AUTORES / AUTHORS:  - Rybakova YS; Boldyrev AA

INSTITUCIÓN / INSTITUTION:  - Research Center of Neurology, Russian Academy of Medical Sciences, Moscow, Russia. julie_ribakova@yahoo.com.

RESUMEN / SUMMARY:  - The study was undertaken to determine the mechanism of carnosine effect on cell proliferation. We studied the effect of carnosine and its derivatives on cell cycle progression in cultured rat pheochromocytoma cells (PC-12). It was found that 48-h incubation of PC-12 cells with carnosine in concentrations of 10-50 mM  led to deceleration of cell proliferation, reduction of G0/G1 peak, and accumulation of S- and G2/M-phase cells. Methylation of the carnosine molecule by 1N-position of the imidazole ring potentiated its effect on cell proliferation. Acetylation of the carnosine molecule by free beta-amino group attenuated its effect.

 

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[65]

TÍTULO / TITLE:  - Photodynamic therapy for bronchial carcinoid tumours: complete response over a 10-year follow-up.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Eur J Cardiothorac Surg. 2013 Jan 2.

            ●● Enlace al texto completo (gratuito o de pago) 1093/ejcts/ezs660

AUTORES / AUTHORS:  - Moghissi K; Dixon K; Gibbins S

INSTITUCIÓN / INSTITUTION:  - The Yorkshire Laser Centre, Goole, UK.

RESUMEN / SUMMARY:  - A 63-year old woman diagnosed in September 2001 with a typical bronchial carcinoid of the left upper lobe bronchus extending into the left main stem bronchus is presented. The patient was unsuitable for standard surgical treatment, and the topography was not amenable for a parenchyma-saving bronchoplastic procedure. Two cycles of bronchoscopic photodynamic therapy (PDT)  were undertaken at 6 monthly intervals. The patient has now been followed up regularly for over 10 years without signs of recurrence bronchoscopically or radiologically. To our knowledge, this is the first case of a carcinoid tumour treated solely by PDT.

 

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[66]

TÍTULO / TITLE:  - Pheochromocytoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Clin Chem. 2012 Dec 3.

            ●● Enlace al texto completo (gratuito o de pago) 1373/clinchem.2012.182246

AUTORES / AUTHORS:  - Estey MP; Diamandis EP; Eisenhofer G; Pacak K; Maher ER; Young WF; de Krijger RR

INSTITUCIÓN / INSTITUTION:  - Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada;

 

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[67]

TÍTULO / TITLE:  - Bladder cancer and seroreactivity to BK, JC and merkel cell polyomaviruses: The spanish bladder cancer study.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Int J Cancer. 2013 Jan 25. doi: 10.1002/ijc.28053.

            ●● Enlace al texto completo (gratuito o de pago) 1002/ijc.28053

AUTORES / AUTHORS:  - Robles C; Viscidi R; Malats N; Silverman DT; Tardon A; Garcia-Closas R; Serra C; Carrato A; Herranz J; Lloreta J; Rothman N; Real FX; de Sanjose S; Kogevinas M

INSTITUCIÓN / INSTITUTION:  - Unit of Infections and Cancer (UNIC), Cancer Epidemiology Research Programme, Catalan Institute of Oncology, IDIBELL, 08908 L’Hospitalet de Llobregat, Barcelona, España.

RESUMEN / SUMMARY:  - An infectious etiology for bladder cancer has long been suspected. Merkel cell virus (MCV), BKV and JCV polyomaviruses are possible causative agents but data remain scarce. Therefore, we evaluated the seroresponse to these three polyomaviruses in association with bladder cancer risk. 1135 incident bladder cancer subjects from 5 Spanish regions and 982 hospital controls matched by sex,  age and region were included. 99% of cases were urothelial-cell carcinomas. Antibody response against MCV, BKV and JCV was measured by enzyme immunoassay using Virus-Like-Particles. Our results show a similar seroprevalence in cases and controls: 64%/60% for BKV, 83%/82% for MCV, and 87%/83% for JCV. However, among seropositive subjects, higher median seroreactivities were observed in cases compared to controls for BKV (0.84 vs. 0.70, p-value=0.009) and MCV (1.81 vs. 0.65, p-value<0.001). Increased bladder cancer risk was observed for BKV (OR=1.4, 95%CI 1.04-1.8) and for MCV (OR=1.5, 95%CI 1.2-1.9), when comparing highest to lowest seroreactivity tertiles. The associations of BKV and MCV with bladder cancer were independent of each other and neither smoking status nor disease stage and grade modified them. Furthermore, no association was observed between seroresponse to JCV and bladder cancer. Therefore, we conclude that BKV and MCV polyomavirus infection could be related to an increased bladder cancer risk.

 

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[68]

TÍTULO / TITLE:  - The Value of Preoperative Imaging in Small Bowel Neuroendocrine Tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Ann Surg Oncol. 2013 Jan 3.

            ●● Enlace al texto completo (gratuito o de pago) 1245/s10434-012-2836-y

AUTORES / AUTHORS:  - Dahdaleh FS; Lorenzen A; Rajput M; Carr JC; Liao J; Menda Y; O’Dorisio TM; Howe JR

INSTITUCIÓN / INSTITUTION:  - Division of Surgical Oncology and Endocrine Surgery, Department of Surgery, University of Iowa Hospitals and Clinics, Iowa City, IA, USA.

RESUMEN / SUMMARY:  - BACKGROUND: Neuroendocrine tumors of the small bowel (SBNETs) are a rare but important subgroup of malignancies. Since 30 % of SBNETs present with metastatic  disease, often with an occult primary, preoperative imaging is critical for determining who will benefit most from abdominal exploration. We set out to evaluate the usefulness of the two most commonly performed imaging modalities in  predicting the extent of disease found at exploration in patients with SBNETs. METHODS: A retrospective chart review was performed on patients with SBNETs resected at 1 institution. Data from preoperative computed tomography (CT) scans  were reviewed to determine whether the primary tumor, nodal, or liver metastases  were seen, then compared with intraoperative findings. Results of preoperative somatostatin receptor scintigraphy (SRS) were similarly examined. RESULTS: A total of 62 patients with SBNETs were included. Of these patients, 42 of 62 (68 %) had distant metastases and 48 of 62 (77 %) had nodal metastases at exploration. A total of 56 patients had preoperative CT scans and 47 had SRS. Using CT, a primary tumor was localized to the small bowel in 27 of 56 (48 %) and nodal metastases seen in 33 of 56 (79 %) of cases. SRS found intra-abdominal uptake in 35 of 47 cases (74 %). CONCLUSIONS: CT and SRS are complementary in making the diagnosis of SBNET, with CT giving more precise anatomical detail, while SRS helps to confirm that lesions are NETs and is useful for identifying occult extrahepatic sites of metastatic disease. However, 10-15 % of SBNETs were  not identified by either test preoperatively, and therefore surgical exploration  still plays an important role in making the diagnosis in these patients.

 

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[69]

TÍTULO / TITLE:  - Neuroendocrine Tumors of the Colon and Rectum: Prognostic Relevance and Comparative Performance of Current Staging Systems.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Ann Surg Oncol. 2012 Dec 1.

            ●● Enlace al texto completo (gratuito o de pago) 1245/s10434-012-2746-z

AUTORES / AUTHORS:  - Chagpar R; Chiang YJ; Xing Y; Cormier JN; Feig BW; Rashid A; Chang GJ; You YN

INSTITUCIÓN / INSTITUTION:  - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

RESUMEN / SUMMARY:  - BACKGROUND: With increasing interest in neuroendocrine tumors (NETs), three staging systems for NETs of the colon and rectum have been published. Their prognostic relevance has not been examined and compared in an independent clinical database. METHODS: From the National Cancer Database (NCDB), 5457 patients diagnosed with colorectal neuroendocrine tumor (CRNETs) between 1998 and 2002 were staged according to the staging systems from (1) European Neuroendocrine Tumor Society (ENETS, 2006; n = 1537); (2) American Joint Committee on Cancer (AJCC, 2009; n = 1140); and (3) location-specific staging systems from the Surveillance Epidemiology and End Results (SEER, 2008; n = 942). Stage-stratified overall survival (OS) and Cox-specific concordance indices were  calculated for each system. Independent prognostic factors were identified by multivariate analysis. RESULTS: Five-year OS for stage I, II, III, and IV CRNETs  as defined by the ENETS staging system were 90.8, 77.3, 53.1, and 14.8 %, respectively. For well-differentiated CRNETs, the 5-year OS for stage I, II, III, and IV as defined by the AJCC staging system were superior: 90.6, 83.9, 64.8, and 24.9 %, respectively. Both staging systems had a concordance index of 0.72. After specifying location in the colon versus rectum, all three systems demonstrated acceptable performance. Histologic grade was a significant independent predictor  of OS not currently incorporated in the staging systems. CONCLUSIONS: The three staging systems showed comparable prognostic stratification of CRNETs, while the  AJCC and ENETS systems are the most parsimonious. The current analysis supports the use of the AJCC for well-differentiated disease and ENETS systems for all CRNETs until there is further evidence for modification.

 

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[70]

TÍTULO / TITLE:  - Cannabinoid CB(1) receptor is expressed in chromophobe renal cell carcinoma and renal oncocytoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Clin Biochem. 2013 Jan 12. pii: S0009-9120(13)00006-4. doi: 10.1016/j.clinbiochem.2012.12.023.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.clinbiochem.2012.12.023

AUTORES / AUTHORS:  - Larrinaga G; Sanz B; Blanco L; Perez I; Candenas ML; Pinto FM; Irazusta A; Gil J; Lopez JI

INSTITUCIÓN / INSTITUTION:  - Department of Physiology, Faculty of Medicine and Dentistry, University of the Basque Country (UPV/EHU), Leioa, Bizkaia, España; Department of Nursing I, Nursing School, University of the Basque Country (UPV/EHU), Leioa, Bizkaia, España. Electronic address: gorka.larrinaga@ehu.es.

RESUMEN / SUMMARY:  - OBJECTIVE: To analyze the mRNA and protein expression of cannabinoid receptors CB1 and CB2 in chromophobe renal cell carcinoma (ChRCC) and renal oncocytoma (RO). DESIGN AND METHODS: Fresh and formalin-fixed tissue samples of ChRCC and RO were analyzed by using real-time quantitative RT-PCR and immunohistochemical techniques (n=40). RESULTS: Quantitative RT-PCR analysis showed that CB1 mRNA was underexpressed by 12-fold in ChRCC and had a variable expression in RO. CB1 protein showed intense positive immunostaining in both neoplasms. Both CB2 mRNA and protein were not expressed in tumor and non tumor renal tissue. CONCLUSION: This distinct immunoprofile may eventually be used as an additional tool with practical interest in the differential diagnosis of renal tumors.

 

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[71]

TÍTULO / TITLE:  - Prognostic factors in Merkel cell carcinoma: Analysis of 240 cases.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Am Acad Dermatol. 2012 Nov 27. pii: S0190-9622(12)01041-9. doi: 10.1016/j.jaad.2012.09.036.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.jaad.2012.09.036

AUTORES / AUTHORS:  - Tarantola TI; Vallow LA; Halyard MY; Weenig RH; Warschaw KE; Grotz TE; Jakub JW; Roenigk RK; Brewer JD; Weaver AL; Otley CC

INSTITUCIÓN / INSTITUTION:  - Department of Dermatology, Mayo Clinic, Rochester, Minnesota. Electronic address: tinatarantola@hotmail.com.

RESUMEN / SUMMARY:  - BACKGROUND: Knowledge regarding behavior of and prognostic factors for Merkel cell carcinoma (MCC) is limited. OBJECTIVE: We sought to further understand the characteristics, behavior, prognostic factors, and optimal treatment of MCC. METHODS: A multicenter, retrospective, consecutive study of patients with known primary MCC was completed. Overall survival and survival free of locoregional recurrence were calculated and statistical analysis of characteristics and outcomes was performed. RESULTS: Among the 240 patients, the mean age at diagnosis was 70.1 years, 168 (70.0%) were male, and the majority was Caucasian.  The most common location was head and neck (111, 46.3%). Immunosuppressed patients had significantly worse survival, with an overall 3-year survival of 43.4% compared with 68.1% in immunocompetent patients. In our study, patients with stage II disease had improved overall survival versus those with stage I disease, in a statistically significant manner. Patients with stage III disease had significantly worse survival compared with stage I and with stage II. Primary tumor size did not predict nodal involvement. CONCLUSION: The data presented represent one of the largest series of primary MCC in the literature and confirm  that MCC of all sizes has metastatic potential, supporting sentinel lymph node biopsy for all primary MCC. Because of the unpredictable natural history of MCC,  we recommend individualization of care based on the details of each patient’s tumor and clinical presentation.

 

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[72]

TÍTULO / TITLE:  - Detection of Merkel cell polyomavirus with a tumour-specific signature in non-small cell lung cancer.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Br J Cancer. 2013 Jan 15. doi: 10.1038/bjc.2012.567.

            ●● Enlace al texto completo (gratuito o de pago) 1038/bjc.2012.567

AUTORES / AUTHORS:  - Hashida Y; Imajoh M; Nemoto Y; Kamioka M; Taniguchi A; Taguchi T; Kume M; Orihashi K; Daibata M

INSTITUCIÓN / INSTITUTION:  - Department of Microbiology and Infection, Kochi Medical School, Kochi University, Nankoku, Kochi 783-8505, Japan.

RESUMEN / SUMMARY:  - Background:We searched for a viral aetiology for non-small cell lung cancer (NSCLC), focusing on Merkel cell polyomavirus (MCPyV).Methods:We analysed 112 Japanese cases of NSCLC for the presence of the MCPyV genome and the expressions  of RNA transcripts and MCPyV-encoded antigen. We also conducted the first analysis of the molecular features of MCPyV in lung cancers.Results:PCR revealed  that 9 out of 32 squamous cell carcinomas (SCCs), 9 out of 45 adenocarcinomas (ACs), 1 out of 32 large-cell carcinomas, and 1 out of 3 pleomorphic carcinomas were positive for MCPyV DNA. Some MCPyV DNA-positive cancers expressed large T antigen (LT) RNA transcripts. Immunohistochemistry showed that MCPyV LT antigen was expressed in the tumour cells. The viral integration sites were identified in one SCC and one AC. One had both episomal and integrated/truncated forms. The other carried an integrated MCPyV genome with frameshift mutations in the LT gene.Conclusion:We have demonstrated the expression of a viral oncoprotein, the presence of integrated MCPyV, and a truncated LT gene with a preserved retinoblastoma tumour-suppressor protein-binding domain in NSCLCs. Although the viral prevalence was low, the tumour-specific molecular signatures support the possibility that MCPyV is partly associated with the pathogenesis of NSCLC in a subset of patients.British Journal of Cancer advance online publication, 15 January 2013; doi:10.1038/bjc.2012.567 www.bjcancer.com.

 

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[73]

TÍTULO / TITLE:  - Malignant insulinoma misdiagnosed and treated as epilepsy.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Ann Endocrinol (Paris). 2013 Jan 22. pii: S0003-4266(12)01208-5. doi: 10.1016/j.ando.2012.11.002.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.ando.2012.11.002

AUTORES / AUTHORS:  - Louda F; Chadli A; Elaziz S; Elghomari H; Farouqi A

INSTITUCIÓN / INSTITUTION:  - Department of Endocrinology and Diabetology, University Hospital Ibn Rochd, Riad  2 No. 198, El Alia Mohammedia 002010, Casablanca, Morocco. Electronic address: fatimalouda@yahoo.fr.

RESUMEN / SUMMARY:  - Pancreatic neuroendocrine tumors (PNET) are extremely rare, and although insulinomas are the commonest, less than 10% of insulinomas are malignant. Most patients with insulinomas present neuroglycopenic symptoms. Because of the rarity of the condition, we report the case of a 56-year-old man with malignant insulinoma, which was misdiagnosed as epilepsy. Timely diagnosis of this disease  is of paramount importance to prevent neurologic sequelae of hypoglycemia. Insulinomas should be regarded from the beginning as potentially malignant, although the majority of malignant insulinomas progresses slowly.

 

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[74]

TÍTULO / TITLE:  - Long-Term Outcome of Laparoscopic Surgery for Pancreatic Neuroendocrine Tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - World J Surg. 2012 Dec 22.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s00268-012-1893-5

AUTORES / AUTHORS:  - Haugvik SP; Marangos IP; Rosok BI; Pomianowska E; Gladhaug IP; Mathisen O; Edwin B

INSTITUCIÓN / INSTITUTION:  - Department of Hepato-Pancreato-Biliary Surgery, Rikshospitalet, Oslo University Hospital, Sognsvannsveien 20, 0372, Oslo, Norway, svhaug@ous-hf.no.

RESUMEN / SUMMARY:  - BACKGROUND: As most pancreatic neuroendocrine tumors (PNET) are relatively small  and solitary, they may be considered well suited for removal by a minimally invasive approach. There are few large series that describe laparoscopic surgery  for PNET. The primary aim of this study was to describe the feasibility, outcome, and histopathology associated with laparoscopic pancreatic surgery (LS) of PNET in a large series. METHODS: All patients with PNET who underwent LS at a single hospital from March 1997 to April 2011 were included retrospectively in the study. RESULTS: A total of 72 patients with PNET underwent 75 laparoscopic procedures, out of which 65 were laparoscopic resections or enucleations. The median operative time of all patients who underwent resections or enucleations was 175 (60-520) min, the median blood loss was 300 (5-2,700) ml, and the median  length of hospital stay was 7 (2-27) days. The overall morbidity rate was 42 %, with a surgical morbidity rate of 21 % and postoperative pancreatic fistula (POPF) formation in 21 %. Laparoscopic enucleations were associated with a higher rate of POPF than were laparoscopic resections. Five-year disease-specific survival rate was 90 %. The T stage, R stage, and a Ki-67 cutoff value of 5 % significantly predicted 5-year survival. CONCLUSION: LS of PNET is feasible with  acceptable morbidity and a good overall disease-specific long-term prognosis.

 

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[75]

TÍTULO / TITLE:  - Primary neuroendocrine tumors of the kidney: morphological and molecular alterations of an uncommon malignancy.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Hum Pathol. 2012 Nov 28. pii: S0046-8177(12)00314-0. doi: 10.1016/j.humpath.2012.08.013.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.humpath.2012.08.013

AUTORES / AUTHORS:  - Aung PP; Killian K; Poropatich CO; Linehan WM; Merino MJ

INSTITUCIÓN / INSTITUTION:  - Translational Surgical Pathology, Laboratory of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA.

RESUMEN / SUMMARY:  - Primary neuroendocrine (NE) tumors of the kidney (PNRTs) are rare and frequently  mistaken for other renal and urothelial cancers. We evaluated morphological and molecular findings of 11 PNRTs classified according to the World Health Organization classification of lung NE tumors. Patients included 5 men and 6 women with a median age of 50 years. These tumors occurred in the left (5/11), right (3/11), and horseshoe (1/11) kidney. The histologic patterns were predominantly solid, trabecular, and pseudoglandular. Lymphovascular invasion and calcification were found in 3 and 1 cases, respectively. There were 2 atypical and 9 typical carcinoids. At the time of surgery, 2 patients with atypical carcinoids had hepatic metastasis, and 1 of the typical carcinoid patients had lymph node metastasis. All cases showed <1% proliferative rate, except 2 cases with hepatic metastasis, which showed 3% to 5% with MIB1/Ki-67 immunostaining. Immunostainings were frequently positive for synaptophysin, chromogranin, CD56, CD99, and neuron-specific enolase. Follow-up data (average 4 years) were available for 6 patients. Two patients with distant metastasis were alive with disease, and four patients with no metastasis were alive without disease. We evaluated the association of PNRT and loss of heterozygosity (LOH) on chromosome  3p21 and found LOH in 2 of 3 cases. However, the comparative genomic hybridization study (2/2) did not demonstrate significant chromosomal imbalances. We conclude that PNRTs are positive for NE markers and may have LOH on chromosome 3p21. PNRTs should be classified as NE tumors in other sites, and proliferative rate can be an indicator of aggressive behavior/metastasis.

 

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[76]

TÍTULO / TITLE:  - Global microRNA profiling of well-differentiated small intestinal neuroendocrine  tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Mod Pathol. 2013 Jan 18. doi: 10.1038/modpathol.2012.216.

            ●● Enlace al texto completo (gratuito o de pago) 1038/modpathol.2012.216

AUTORES / AUTHORS:  - Li SC; Essaghir A; Martijn C; Lloyd RV; Demoulin JB; Oberg K; Giandomenico V

INSTITUCIÓN / INSTITUTION:  - Department of Medical Sciences, Uppsala University, Uppsala, Sweden.

RESUMEN / SUMMARY:  - Well-differentiated small intestinal neuroendocrine tumors are rare malignancies. They arise from enterochromaffin cells and very little is known about differential microRNA (miRNA) expression. The aim of this study was to identify the miRNA profile of well-differentiated small intestinal neuroendocrine tumors,  which may have a critical role in tumor development, progression and potentially  develop miRNAs as novel clinical biomarkers. Specimens from two test groups, 24 small intestinal neuroendocrine tumor specimens at different stages of malignancy, are included in this study. Total RNA from the first test group, five primary tumors, five mesentery metastases and five liver metastases was hybridized onto the Affymetrix Genechip miRNA arrays to perform a genome-wide profile. The results were validated by using quantitative real-time PCR (QRT-PCR) and northern blot analyses. We then expanded the investigation to laser capture microdissected small intestinal neuroendocrine tumor cells and immuno-laser capture microdissected normal enterochromaffin cells of the first test group. Furthermore, a second test group, three primary tumors, three mesentery metastases and three liver metastases, was included in the study. Thus, two independent test groups validated the data by QRT-PCR. Moreover, we characterized nine miRNAs, five (miR-96, -182, -183, -196a and -200a), which are upregulated during tumor progression, whereas four (miR-31, -129-5p, -133a and -215) are downregulated. Several online software programs were used to predict potential miRNA target genes to map a number of putative target genes for the aberrantly regulated miRNAs, through an advanced and novel bioinformatics analysis. Our findings provide information about pivotal miRNAs, which may lead to further insights into tumorigenesis, progression mechanisms and novel therapeutic targets recognition.Modern Pathology advance online publication, 18 January 2013; doi:10.1038/modpathol.2012.216.

 

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[77]

TÍTULO / TITLE:  - Review of renal carcinoid tumor with focus on clinical and pathobiological aspects.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Histol Histopathol. 2013 Jan;28(1):15-21.

AUTORES / AUTHORS:  - Kuroda N; Tanaka A; Ohe C; Mikami S; Nagashima Y; Inoue K; Shuin T; Taguchi T; Tominaga A; Alvarado-Cabrero I; Petersson F; Brunelli M; Martignoni G; Michal M; Hes O

INSTITUCIÓN / INSTITUTION:  - Department of Diagnostic Pathology, Kochi Red Cross Hospital, Tokyo, Japan. kurochankochi@yahoo.co.jp

RESUMEN / SUMMARY:  - Renal carcinoid tumor is a rare neoplasm. In this article, we review this neoplasm with a focus on clinical and pathobiological aspects. The majority of patients present in the fourth to seventh decades, but there is no gender predilection. Clinically, patients with renal carcinoid tumor frequently present  with abdominal, back or flank pain. This tumor is occassionally associated with horseshoe kidney and/or mature cystic teratoma located in the kidney. Macroscopically, these tumors are well demarcated with a lobulated appearance and yellow or tan-brown color cut surface. Microscopically, these tumors are composed of monomorphic round to polygonal cells with granular amphophilic to eosinophilic cytoplasm. Tumor cells are arranged in trabecular, ribbon-like, gyriform, insular, glandular and solid patterns. The nuclei are round to oval and with evenly distributed nuclear chromatin, frequently with a “salt and pepper”-pattern. Immunohistochemically, tumor cells demonstrate immuno-labeling for chromogranin A and synaptophysin. Ultrastructurally, the neoplastic cells contain abundant dense core neurosecretory granules. In previous genetic studies, abnormalities of chromosomes 3 or 13 have been reported. The clinical behavior of renal carcinoid tumors is variable, but is more indolent than most renal cell carcinomas. Further investigations are warranted in order to elucidate the critical genetic abnormalities responsible for the pathogenesis of this rare entity in renal neoplastic pathology.

 

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[78]

TÍTULO / TITLE:  - Surgical management of a neuroendocrine tumor of the inferior vena cava.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Vasc Surg. 2013 Jan 24. pii: S0741-5214(12)02439-1. doi: 10.1016/j.jvs.2012.10.132.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.jvs.2012.10.132

AUTORES / AUTHORS:  - Ransford G; Ayyathurai R; Fernandez G; Ciancio G

INSTITUCIÓN / INSTITUTION:  - University of Miami, Miller School of Medicine, Miami, Fla. Electronic address: gransford@med.miami.edu.

RESUMEN / SUMMARY:  - Neuroendocrine tumors occurring outside of the gastrointestinal tract or lungs are very few, and to find a primary neuroendocrine tumor of the infrahepatic inferior vena cava (IVC) is extremely rare. We present a case of a patient with a large, 7 x 4 cm neuroendocrine tumor of the IVC, where the IVC and renal veins were completely extirpated and not reconstructed. As a result, the liver was anastomosed to the intrapericardial IVC, and the patient relied on collateral drainage. After an initial postoperative period of anasarca and weight gain, she  ultimately recovered fully with no evidence of recurrence in the IVC.

 

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[79]

TÍTULO / TITLE:  - Merkel Cell Carcinoma: 30-Year Experience from a Single Institution.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Ann Surg Oncol. 2012 Dec 1.

            ●● Enlace al texto completo (gratuito o de pago) 1245/s10434-012-2779-3

AUTORES / AUTHORS:  - Santamaria-Barria JA; Boland GM; Yeap BY; Nardi V; Dias-Santagata D; Cusack JC Jr

INSTITUCIÓN / INSTITUTION:  - Department of Surgery, Massachusetts General Hospital, Harvard Medical School, YAW 7B, 55 Fruit Street, Boston, MA, 02114, USA.

RESUMEN / SUMMARY:  - BACKGROUND: Merkel cell carcinoma (MCC) is a rare cutaneous malignancy. Few single-institution series have been reported. METHODS: Review of MCC patients treated at our institution between 1980 and 2010. Patient, tumor, and treatment variables were analyzed to determine MCC-specific outcomes. RESULTS: We identified 161 patients with MCC. There was a 2.5-fold increase in cases over the last decade. Median length of follow-up was 36 months. Stage at diagnosis was I in 35 %, II in 21 %, IIIa in 12 %, IIIb in 23 %, and IV in 9 %. The 5-year MCC-specific survival rates were 87, 63, 42, and 0 % for stages I, II, III, and IV, respectively. Death from the disease occurred in 10 % of patients with T1 and in 50 % with larger lesions. One-third of patients presented with nodal disease.  Sentinel lymph node biopsy (SLNB) identified micrometastases in 9 out of 27 (33 %) early-stage patients. Recurrence developed in 56 % of SLNB-positive and 39 % of SLNB-negative patients. Half of patients recurred after a median time of 9 months. Proportions of first recurrence location were distant (52 %), nodal (27 %), and local (21 %). Adjuvant treatments did not improve recurrence or survival  rates. One-third of patients died of the disease. CONCLUSIONS: SLNB identifies micrometastasis in one-third of early-stage patients. Negative SLNB may predict for improved but not necessarily favorable outcome. Initial tumor size and clinical nodal disease predict for poor outcome. High recurrence rates warrant the development of more effective adjuvant therapies, and better markers of recurrence and treatment response for MCC are needed.

 

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[80]

TÍTULO / TITLE:  - Merkel cell polyomavirus: a newly discovered human virus with oncogenic potential.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Virology. 2013 Jan 5;435(1):118-30. doi: 10.1016/j.virol.2012.09.029.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.virol.2012.09.029

AUTORES / AUTHORS:  - Spurgeon ME; Lambert PF

INSTITUCIÓN / INSTITUTION:  - Department of Oncology, McArdle Laboratory for Cancer Research, University of Wisconsin School of Medicine and Public Health, Madison, WI 53706, USA.

RESUMEN / SUMMARY:  - A marked escalation in the rate of discovery of new types of human polyomavirus has occurred over the last five years largely owing to recent technological advances in their detection. Among the newly discovered viruses, Merkel Cell Polyomavirus (MCPyV or MCV) has gained the most attention due to its link with a  rare human cancer. Infection with MCPyV is common in the human population, and the virus is detected in several anatomical locations, but most frequently in skin. Study of MCPyV molecular virology has been complicated by the lack of straightforward cell culture models, but recent in vitro studies are making strides towards understanding the virus life cycle, its cellular tropism, and mode of transmission. While MCPyV shares several traditional traits with other human polyomaviruses, the burst of research since its discovery reveals insight into a virus with many unique genetic and mechanistic features. The evidence for  a causal link between MCPyV and the rare neuroendocrine cancer, Merkel Cell Carcinoma (MCC), is compelling. A majority of MCCs contain clonally integrated viral DNA, express viral T antigen transcripts and protein, and exhibit an addiction to the viral large T and small t antigen oncoproteins. The MCPyV large  T antigen contains MCC tumor-specific mutations that ablate its replication capacity but preserve its oncogenic functions, and the small t antigen promotes an environment favorable for cap-dependent translation. The mechanisms of MCPyV-induced transformation have not been fully elucidated, but the likely etiological role of this new polyomavirus in human cancer provides a strong opportunity to expand knowledge of virus-host interactions and viral oncology.

 

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[81]

TÍTULO / TITLE:  - Paraganglioma of the urinary bladder—clinicopathological, immunohistochemical and electron microscopy analysis—a case report.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Coll Antropol. 2012 Sep;36(3):1041-3.

AUTORES / AUTHORS:  - Persec Z; Bukovic D; Persec J; Sovic T; Ljubanovic D; Lambasa S; Radan M; Babic I

INSTITUCIÓN / INSTITUTION:  - University of Zagreb, Dubrava University Hospital, Department of Urology, Zagreb, Croatia. zpersec@net.amis.hr

RESUMEN / SUMMARY:  - Tumors that grow within the adrenal medulla are called pheochromocytoma; when located extra-adrenal, they are called paraganglioma. Paraganglioma of the bladder are very rare, with only 180 reported cases. Less than 30 were malignant. We report a case of a 72-years old man with bladder paraganglioma who presented with painless hematuria. Urgent transurethral resection (TUR) was performed. Definitive pathohistological diagnosis was confirmed to imunohistochemical and electron microscopy. Clinical diagnostic showed normal value of epinephrine and norepinehrine in the urine. Scintigraphy of entire body and targeted pictures of  pelvis where taken 24, 48 and 72 hours after administration of RI. No loci of pathologic accumulation of 131-I MIBG where found. Computer tomography (CT) of pelvis and abdomen were normal. Considering staging and pathohistological analysis, we treated our patient with TUR and longtime follow-up afterworth.

 

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[82]

TÍTULO / TITLE:  - Medullary breast carcinoma in an 18-year-old female report on one case diagnosed  on fine-needle cytology sample.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Diagn Cytopathol. 2013 Jan 22. doi: 10.1002/dc.22947.

            ●● Enlace al texto completo (gratuito o de pago) 1002/dc.22947

AUTORES / AUTHORS:  - Galzerano A; Rocco N; Accurso A; Ciancia G; Campanile AC; Caccavello F; Fulciniti F

INSTITUCIÓN / INSTITUTION:  - Servico de Anatomia Patologica, Centro Hospitalar de Lisboa Ocidental, Lisbon, Portugal.

RESUMEN / SUMMARY:  - Medullary breast carcinoma (MBC) is a rare epithelial malignancy of the breast accounting for about 1-7% of all breast carcinomas. It is characterized by well-defined borders, a syncytial/solid pattern of growth of high grade atypical  cells showing no glandular differentiation and a massive diffuse lympho-plasmacytic peritumoral infiltrate. Despite the high-grade atypias characterizing this neoplasm, MBC has been reported to have a better prognosis when compared with the common infiltrating duct carcinoma. MBCs typically lack estrogen and progesterone receptor (ER and PgR) expression and have a low incidence of ERBB2 overexpression. Genetically, they are often associated with BRCA-1 oncogene mutations and TP53 alterations. While MBC generally occurs in middle-aged women, ranging from 45 to 52 years of age, we report the case of a 18-year-old female patient which was diagnosed by means of fine-needle cytology sample. © 2013 Wiley Periodicals, Inc.

 

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[83]

TÍTULO / TITLE:  - Neurological manifestations of phaeochromocytomas and secretory paragangliomas: a reappraisal.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Neurol Neurosurg Psychiatry. 2012 Dec 1.

            ●● Enlace al texto completo (gratuito o de pago) 1136/jnnp-2012-303028

AUTORES / AUTHORS:  - Anderson NE; Chung K; Willoughby E; Croxson MS

INSTITUCIÓN / INSTITUTION:  - Department of Neurology, Auckland Hospital, , Auckland, New Zealand.

RESUMEN / SUMMARY:  - OBJECTIVE: To determine the frequency and range of neurological manifestations of phaeochromocytomas and secretory paragangliomas. METHODS: A retrospective review  of case notes of patients admitted to Auckland Hospital from 1985 to 2011 with a  discharge diagnosis of phaeochromocytoma or secretory paraganglioma. RESULTS: Ninety-three patients were admitted with a phaeochromocytoma or secretory paraganglioma. Sixty-eight patients (73%) had neurological symptoms, but only 15  patients (16%) received a neurological consultation. Neurological manifestations  occurred in three main clinical contexts. First, paroxysmal symptoms occurred in  66 of 93 patients (71%). Neurological symptoms were common features of these attacks and included headache (47 patients), anxiety (24 patients), tremulousness (15 patients) and dizziness (12 patients). The headaches typically had an explosive onset. Delay in diagnosis was common. Second, 28 patients (30%) had an  acute crisis, which was associated with neurological symptoms in 11 (39%) of the  episodes: headache (10 patients); seizures (five patients); strokes (three patients); delirium (three patients) and subarachnoid haemorrhage (one patient).  Third, five of six patients with a head and neck secretory paraganglioma had neurological symptoms related to infiltration of the middle ear or compression of cranial nerves. Reversible cerebral vasoconstriction syndrome (RCVS) was documented in three patients. CONCLUSIONS: Neurological manifestations of phaeochromocytomas and secretory paragangliomas were common, and these tumours can present with various neurological manifestations. The paroxysmal symptoms can be incorrectly attributed to other headache syndromes, panic attacks or cerebral  vasculitis. RCVS may play a role in the pathogenesis of the neurological symptoms associated with acute crises and paroxysmal attacks.

 

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[84]

TÍTULO / TITLE:  - Evidence of a Low Prevalence of RAS Mutations in a Large Medullary Thyroid Cancer Series.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Thyroid. 2013 Jan;23(1):50-7. doi: 10.1089/thy.2012.0207. Epub 2012 Dec 16.

            ●● Enlace al texto completo (gratuito o de pago) 1089/thy.2012.0207

AUTORES / AUTHORS:  - Ciampi R; Mian C; Fugazzola L; Cosci B; Romei C; Barollo S; Cirello V; Bottici V; Marconcini G; Rosa PM; Borrello MG; Basolo F; Ugolini C; Materazzi G; Pinchera A; Elisei R

INSTITUCIÓN / INSTITUTION:  - 1 Department of Endocrinology and Metabolism, University Hospital of Pisa , Italy .

RESUMEN / SUMMARY:  - Background: Approximately 60% of sporadic medullary thyroid carcinomas (sMTC) remain orphan of a recognized genetic cause. Recently, a high percentage of RAS point mutations have been described in RET-negative sMTC. The aim of this study was to assess the prevalence of RAS point mutations in a large series of MTC collected in four Italian centers. Methods: For this purpose, we studied codons 12, 13, and 61 of H-, K-, and N-RAS genes in 188 MTC samples, either hereditary or sporadic, by direct sequencing. Correlations between the RAS mutational status and the clinical-pathological features of MTC patients as well as a meta-analysis of all published data were performed. Results: The prevalence of RAS mutations in the present series of MTC was 10.1%, and 17.6% when considering only RET-negative cases. RAS mutations were found in MTC tumoral tissue, but not in peripheral blood indicating their somatic origin. A novel mutation in codon 72 (M72I) was found, but with a low or null transforming potential. No association was found between the presence of RAS mutations and the clinical-pathological features of the patients. Although not statistically significant, a positive association between the presence of RAS mutations and a better outcome was observed. The meta-analysis of all published studies confirmed a prevalence of 8.8% for RAS mutations in MTC. Conclusions: The prevalence of RAS mutations in our MTC series  was relatively low and consistent with the meta-analysis data. Only somatic RAS mutations were found and only in RET-negative sMTC. Likewise, MTCs that harbor a  RAS mutation identify a subgroup of tumors with less aggressive behavior. To our  knowledge, this is the largest series of MTCs studied for the presence of mutations in RAS genes and the first meta-analysis on this specific topic.

 

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[85]

TÍTULO / TITLE:  - Diagnostic utility of MS-MLPA in DNA methylation profiling of adenocarcinomas and neuroendocrine carcinomas of the colon-rectum.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Virchows Arch. 2013 Jan;462(1):47-56. doi: 10.1007/s00428-012-1348-2. Epub 2012 Dec 9.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s00428-012-1348-2

AUTORES / AUTHORS:  - Furlan D; Sahnane N; Mazzoni M; Pastorino R; Carnevali I; Stefanoli M; Ferretti A; Chiaravalli AM; La Rosa S; Capella C

INSTITUCIÓN / INSTITUTION:  - Department of Surgical and Morphological Sciences, Anatomic Pathology Unit, University of Insubria and Ospedale di Circolo, Via O. Rossi, 9, 21100, Varese, Italy, daniela.furlan@uninsubria.it.

RESUMEN / SUMMARY:  - Methylation-specific multiple ligation-dependent probe amplification (MS-MLPA) is a fast, new, inexpensive method that has rarely been exploited in DNA methylation profiling of colorectal cancers (CRCs). The aim of this study was to test the diagnostic utility of MS-MLPA to evaluate the methylation status of 34 genes in normal colonic mucosa samples and in a well-characterized series of 83 adenocarcinomas and 21 neuroendocrine carcinomas of colon-rectum. Two commercial  MS-MLPA kits (SALSA MS-MLPA ME001-C1 Tumor suppressor-1 Kit and SALSA MS-MLPA ME002-B1 Tumor suppressor-2 Kit) were used to perform promoter methylation analysis on formalin-fixed and paraffin-embedded tissues. MS-MLPA analysis was validated by bisulfite pyrosequencing, bisulfite cycle sequencing, and methylation-specific PCR. MS-MLPA analysis identified a subset of 27 CRCs (26 % of cases) showing high levels of gene methylation involving a mean percentage of  34 % of the promoters examined. These tumors exhibited all the main clinicopathological and genetic features described for CRCs with CpG island Methylator Phenotype-High. High levels of methylation were observed with similar  frequency in adenocarcinomas and in neuroendocrine carcinomas (25 % versus 29 %,  respectively), but different methylation profiles were observed in the two tumor  types. In both groups, tumors with microsatellite instability and widespread methylation represented a homogeneous clinicopathological entity. MS-MLPA assay is an easy and reliable system for epigenetic characterization of tumor tissues and leads to a rapid identification of CRCs with the highest levels of gene methylation. Aberrant gene methylation is a common abnormality in CRC initiation  and may be observed in tumors with very different genetic and clinicopathological profiles.

 

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[86]

TÍTULO / TITLE:  - Commentary: a gastrin antagonist against carcinoids - implications for PPI-induced hypergastrinaemia.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Aliment Pharmacol Ther. 2013 Jan;37(2):276-7. doi: 10.1111/apt.12135.

            ●● Enlace al texto completo (gratuito o de pago) 1111/apt.12135

AUTORES / AUTHORS:  - McCarthy DM

INSTITUCIÓN / INSTITUTION:  - Division of Gastroenterology University of New Mexico School of Medicine, Veteran’s Administration Medical Center, Albuquerque, NM, USA. dmccarthy@salud.unm.edu.

 

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[87]

TÍTULO / TITLE:  - Antitumor Effect of Everolimus in Preclinical Models of High Grade Gastroenteropancreatic Neuroendocrine Carcinomas.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Neuroendocrinology. 2013 Jan 23.

            ●● Enlace al texto completo (gratuito o de pago) 1159/000347063

AUTORES / AUTHORS:  - Bollard J; Couderc C; Blanc M; Poncet G; Lepinasse F; Hervieu V; Gouysse G; Ferraro-Peyret C; Benslama N; Walter T; Scoazec JY; Roche C

INSTITUCIÓN / INSTITUTION:  - Endocrine Differentiation and Tumorigenesis Laboratory, Centre de Recherche en Cancerologie de Lyon, INSERM U1052-CNRS UMR5286, Universite de Lyon, Faculte Laennec, Lyon, France.

RESUMEN / SUMMARY:  - Background/Aims: While the range of therapeutic options for well-differentiated gastroenteropancreatic neuroendocrine tumors (GEP NETs) has recently increased with the emergence of targeted therapies, such as mTOR inhibitors, there is no recent progress in the treatment of poorly differentiated neuroendocrine carcinomas (PDNECs). Since PDNECs have been shown to strongly express mTOR pathway components, the aim of the present study was to assess the antitumor effect of the mTOR inhibitor everolimus in preclinical models of PDNECs. Methods: Thee expression of mTOR pathway components and their response to everolimus were  assessed in two neuroendocrine cell lines: STC-1 and GluTag. A xenograft model of intra-hepatic dissemination in the nude mouse, based on the intrasplenic injection of either STC-1 and GluTag tumor cells, was used. Animals were started  on everolimus treatment 3 days after injection. The effects of treatment on tumor growth, proliferative capacities, apoptosis and in situ expression of mTOR pathway components were assessed. Results: The expression of mTOR pathway components was comparable in STC-1 and GluTag cells and in human PDNECs and could be inhibited in vitro by everolimus. In vivo, the tumor volume of STC-1 and GluTag xenografts was significantly reduced in treated animals (6.05% +/- 1.84 as compared to 21.76 +/- 3.88% in controls). Everolimus treatment also induced a significant decrease in Ki67 index and in the phosphorylation levels of the two major effectors of mTOR, p70S6K and 4E-BP1. Conclusion: Our experimental data suggest that mTOR inhibition could be considered a therapeutic option for high grade GEP NETs.

 

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[88]

TÍTULO / TITLE:  - Functional characterization of non-metastatic paraganglioma and pheochromocytoma  by (18) F-FDOPA PET: focus on missed lesions.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Clin Endocrinol (Oxf). 2012 Dec 11. doi: 10.1111/cen.12126.

            ●● Enlace al texto completo (gratuito o de pago) 1111/cen.12126

AUTORES / AUTHORS:  - Gabriel S; Blanchet EM; Sebag F; Chen CC; Fakhry N; Deveze A; Barlier A; Morange I; Pacak K; Taieb D

INSTITUCIÓN / INSTITUTION:  - Department of Nuclear Medicine, La Timone University Hospital, CERIMED, Aix-Marseille University, Marseille, France.

RESUMEN / SUMMARY:  - AIMS AND METHODS: To evaluate the clinical value of (18) F-fluorodihydroxyphenylalanine ((18) F-FDOPA) PET in relation to tumour localization and the patient’s genetic status in a large series of pheochromocytoma/paraganglioma (PHEO/PGL) patients and to discuss in detail false-negative results. A retrospective study of PGL patients who were investigated with (18) F-FDOPA PET or PET/CT imaging in two academic endocrine tumour centers was conducted (La Timone University Hospital, Marseilles, France and National Institutes of Health (NIH), Bethesda, MD, USA). RESULTS: One hundred sixteen patients (39.7% harboring germline mutations in known disease susceptibility genes) were evaluated for a total of 195 PHEO/PGL foci. (18) F-FDOPA PET correctly detected 179 lesions (91.8%) in 107 patients (92.2%). Lesion-based sensitivities for parasympathetic PGLs (head, neck, or anterior/middle thoracic ones), PHEOs, and extra-adrenal sympathetic (abdominal or posterior thoracic) PGLs were 98.2% [96.5% for Timone and 100% for NIH], 93.9% [93.8% and 93.9%], and 70.3% [47.1% and 90%], respectively (P<0.001). Sympathetic (adrenal and extra-adrenal) SDHx-related PGLs were at a higher risk for negative  (18) F-FDOPA PET than non-SDHx-related PGLs (14/24 vs 0/62, respectively, p<0.001). By contrast, the risk of negative (18) F-FDOPA PET was lower for parasympathetic PGLs regardless of the genetic background (1/90 in SDHx vs 1/19 in non-SDHx tumours, p= 0.32). (18) F-FDOPA PET failed to detect 2 head and neck  PGLs (HNPGL), likely due to their small size, while most missed sympathetic PGL were larger and may have exhibited a specific (18) F-FDOPA-negative imaging phenotype. (18) F-FDG PET detected all the missed sympathetic lesions. CONCLUSIONS: (18) F-FDOPA PET appears to be a very sensitive functional imaging tool for HNPGL regardless of the genetic status of the tumours. Patients with false-negative tumours on (18) F-FDOPA PET should be tested for SDHx mutations. © 2012 Blackwell Publishing Ltd.

 

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[89]

TÍTULO / TITLE:  - Notch 1 tumor expression is lacking in highly proliferative pancreatic neuroendocrine tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endocrine. 2012 Dec 7.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s12020-012-9850-5

AUTORES / AUTHORS:  - Krausch M; Kroepil F; Lehwald N; Lachenmayer A; Schott M; Anlauf M; Cupisti K; Knoefel WT; Raffel A

INSTITUCIÓN / INSTITUTION:  - Department of General, Visceral and Pediatric Surgery, Heinrich-Heine-University  Dusseldorf, Moorenstrasse 5, 40225, Dusseldorf, Germany.

RESUMEN / SUMMARY:  - To date, very little is known about the development of benign organic hyperinsulinism and its metastatic potential. Typical morphologic, biochemical, or genetic differentiations for benign or malign tumor course of insulinomas do not exist. As signaling pathways may affect pancreatic cancer development and the maintenance of the neoplastic phenotype, the purpose of this study was to examine the role of Notch1 expression in organic hyperinsulinism. We examined 32 well-differentiated pancreatic endocrine tumors (wd PET); 11 wd PET of unknown behavior (wd PET ub); and 15 wd pancreatic endocrine cancer (wd PEC) for Notch1 expression by immunohistochemistry. Demographic data, clinical data, and follow-up of all patients were analyzed. Islets of the Langerhans show the strongest Notch1 staining in nearly 90 %. Positive Notch1 staining was absent in  the acinar of the pancreas. In patients with a wd PET more than every second tumor (56.3 %/n = 18/32) demonstrated a negative Notch1 staining. The other 14 patients were positive for Notch1. Tumors of unknown behavior (wd PET ub) and malignant insulinomas had no signs of Notch expression in contrast to benign insulinomas. Considering the clinical and histomorphological tumor behavior, no correlation between Notch1 expression and clinical data was found. The missing Notch expression in the malignant tumor course might be used as a potential predictive marker, but further studies are needed to investigate the underlying molecular mechanism.

 

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[90]

TÍTULO / TITLE:  - Pancreatic Neuroendocrine Tumor Associated With Humoral Hypercalcemia of Malignancy and Carcinoid Tumor: A Case Report and Review of the Literature.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Pancreas. 2012 Dec 1.

            ●● Enlace al texto completo (gratuito o de pago) 1097/MPA.0b013e318267c987

AUTORES / AUTHORS:  - Shah RH; Martinez D

INSTITUCIÓN / INSTITUTION:  - Department of Internal Medicine, UCLA Medical Center, Los Angeles, CA, renashah@mednet.ucla.edu Department of Endocrinology, UCLA Medical Center, Los Angeles, CA.

 

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[91]

TÍTULO / TITLE:  - A reliable and simple method for the assay of neuroendocrine tumor markers in human urine by solid-phase microextraction-gas chromatography-triple quadrupole mass spectrometry.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Anal Chim Acta. 2013 Jan 8;759:66-73. doi: 10.1016/j.aca.2012.11.017. Epub 2012 Nov 23.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.aca.2012.11.017

AUTORES / AUTHORS:  - Monteleone M; Naccarato A; Sindona G; Tagarelli A

INSTITUCIÓN / INSTITUTION:  - Dipartimento di Chimica, Universita della Calabria, Via Pietro Bucci Cubo 12/C, I-87030 Arcavacata di Rende (CS), Italy.

RESUMEN / SUMMARY:  - Homovanillic acid (HVA), vanylmandelic acid (VMA), and 5-hydroxyindoleacetic acid (5-HIAA) are the metabolites of some catecholamines such as epinephrine, nor-epinephrine, dopamine and serotonin and their quantification is used in the diagnosis and management of patients with neurocrine tumors. A novel approach in  the assay of these biomarkers in human urine samples by solid phase microextraction (SPME) combined with gas chromatography-triple quadrupole mass spectrometry (GC-QqQ-MS) is presented. A preliminary derivatization with ethyl chloroformate/ethanol was used and the corresponding derivatives were then extracted by SPME in immersion mode. The performance of five SPME fibers and three chloroformates were evaluated in univariate mode and the best results were  obtained using the polyacrylate fiber and ethyl chloroformate. The variables affecting the efficiency of SPME analysis were optimized by the multivariate approach of “Experimental design” and, in particular, a central composite design  (CCD) was applied. The optimum working conditions in terms of response values were achieved by performing analysis at room temperature with addition of NaCl (9.5%) and with an extraction time of 25.8min. Identification and quantification  of analytes were carried out by using a gas chromatography-triple quadrupole mass spectrometry (GC-QqQ MS) system in multiple reaction monitoring (MRM) acquisition. An evaluation of all analytical parameters shows that the proposed method provides satisfactory results. Very good linearities were, in fact, achieved in the tested calibration ranges with correlation coefficient values >0.99 for all the analytes and accuracies and RSDs calculated for between-run and tested at concentrations of 1, 10, and 80mgL(-1) were ranging from 91.3% to 106.6%, and from 0.5 to 8.9%, respectively. Moreover, the LOD values obtained can be considered very satisfactory (1.3, 0.046 and 24.3mugL(-1) for HVA, VMA and 5-HIAA, respectively). The developed protocol represents, therefore, a simple, rapid and selective tool for assaying these acidic biomarkers in urine samples for neuroendocrine cancer diagnosis.

 

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[92]

TÍTULO / TITLE:  - Twelve cases of neuroendocrine carcinomas of the uterine cervix: cytology, histopathology and discussion of their histogenesis.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Acta Cytol. 2013;57(1):54-60. doi: 10.1159/000342516. Epub 2012 Dec 6.

            ●● Enlace al texto completo (gratuito o de pago) 1159/000342516

AUTORES / AUTHORS:  - Li S; Zhu H

INSTITUCIÓN / INSTITUTION:  - Department of Pathology, Shanghai First Maternity and Infant Hospital, Tongji University School of Medicine, Shanghai, China.

RESUMEN / SUMMARY:  - Objective: Neuroendocrine carcinoma (NEC) of the uterine cervix is a highly aggressive type of tumor. Therefore, recognition of its cytology and histopathology is important for early diagnosis. Study Design: We report 6 cases  of small-cell and 6 cases of large-cell NEC of the uterine cervix, including 5 cytology slides. AE1/3, CEA, p63, p16, CD56, chromogranin and synaptophysin were  detected. Results: Two cytology slides of small-cell NEC showed sheet-like clusters. The tumor cells were uniformly small, with finely granular chromatin, scant cytoplasm and absent nucleoli. Three large-cell NEC smears showed palisading, molding and abortive rosettes with prominent eosinophilic nucleoli. The tumor cells had either abundant cytoplasm or no cytoplasm at all. The histopathology analysis indicated a transition from dysplasia glands or adenocarcinoma in situ glands to NEC in 6 cases. Conclusion: Cervical cytology of NEC has many unique characteristics, distinct from the characteristics of other lesions. We also demonstrated a hypothesis of the histogenesis of some NECs.

 

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[93]

TÍTULO / TITLE:  - Pheochromocytoma crisis resulting in acute heart failure and cardioembolic stroke in a 37-year-old man.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Surgery. 2013 Jan 7. pii: S0039-6060(12)00713-1. doi: 10.1016/j.surg.2012.11.013.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.surg.2012.11.013

AUTORES / AUTHORS:  - Cohen JK; Cisco RM; Scholten A; Mittmaker E; Duh QY

INSTITUCIÓN / INSTITUTION:  - University of California, San Francisco, CA.

 

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[94]

TÍTULO / TITLE:  - Management of exclusively dopamine-secreting abdominal pheochromocytomas.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Am Coll Surg. 2013 Feb;216(2):340-6. doi: 10.1016/j.jamcollsurg.2012.10.002. Epub 2012 Nov 30.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.jamcollsurg.2012.10.002

AUTORES / AUTHORS:  - Poirier E; Thauvette D; Hogue JC

INSTITUCIÓN / INSTITUTION:  - Centre des Maladies du Sein Deschenes-Fabia, Hopital du Saint-Sacrement, CHA Universitaire de Quebec, Quebec City, Quebec, Canada. Electronic address: ricpoirier@me.com.

 

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[95]

TÍTULO / TITLE:  - Pheochromocytoma presenting with bidirectional ventricular tachycardia.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Heart. 2013 Jan 12.

            ●● Enlace al texto completo (gratuito o de pago) 1136/heartjnl-2012-303311

AUTORES / AUTHORS:  - Traykov VB; Kotirkov KI; Petrov IS

INSTITUCIÓN / INSTITUTION:  - Clinic of Cardiology and Angiology, Tokuda Hospital, , Sofia, Bulgaria.

 

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[96]

TÍTULO / TITLE:  - Underexpression of hepatocyte nuclear factor-1beta in chromophobe renal cell carcinoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Histopathology. 2012 Sep 12. doi: 10.1111/his.12026.

            ●● Enlace al texto completo (gratuito o de pago) 1111/his.12026

AUTORES / AUTHORS:  - Wang CC; Mao TL; Yang WC; Jeng YM

INSTITUCIÓN / INSTITUTION:  - Department of Pathology, National Taiwan University Hospital and College of Medicine, National Taiwan University, Taipei, Taiwan.

RESUMEN / SUMMARY:  - AIMS: Chromophobe renal cell carcinoma (ChRCC) is an uncommon malignant renal neoplasm with a generally indolent clinical behaviour. Previous studies revealed  biallelic inactivation of the hepatocyte nuclear factor-1beta (HNF1beta) gene in  several patients with ChRCC. The aims of this study were to determine HNF1beta expression in renal neoplasms and the potential of HNF1beta as a diagnostic marker for ChRCC. METHODS AND RESULTS: We performed immunohistochemical staining  of 79 samples taken from patients with primary renal neoplasm [19 renal oncocytomas, 18 ChRCCs, 24 clear cell renal cell carcinomas (CCRCCs), and 18 papillary renal cell carcinomas]. HNF1beta was underexpressed in 16 of 18 cases of ChRCC (88.9%). By contrast, HNF1beta expression was preserved in the majority  of renal oncocytoma (94.7%, 18/19) and CCRCC (95.8%, 23/24) cases. The combined use of HNF1beta and cytokeratin 7 (CK7) further increased the diagnostic sensitivity and specificity; the profile of HNF1beta positivity and CK7 negativity was not visible in any ChRCC sample, but was common in both renal oncocytoma (94.7%, 18/19) and CCRCC (91.7%, 22/24) samples. CONCLUSIONS: The results suggest that a lack of HNF1beta expression might play an important role in the pathogenesis of ChRCC, and may serve as a good diagnostic marker for this  neoplasm.

 

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[97]

TÍTULO / TITLE:  - Incidental Neuroendocrine Tumors of the Pancreas: MDCT Findings and Features of Malignancy.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - AJR Am J Roentgenol. 2013 Feb;200(2):355-62. doi: 10.2214/AJR.11.8037.

            ●● Enlace al texto completo (gratuito o de pago) 2214/AJR.11.8037

AUTORES / AUTHORS:  - Gallotti A; Johnston RP; Bonaffini PA; Ingkakul T; Deshpande V; Castillo CF; Sahani DV

INSTITUCIÓN / INSTITUTION:  - Department of Radiology, University Hospital “G. B. Rossi,” University of Verona, Verona, Italy.

RESUMEN / SUMMARY:  - OBJECTIVE: The objective of our study was to evaluate the MDCT features of incidentally detected neuroendocrine tumors (NETs) of the pancreas, identify features that can predict tumor biology or aggressiveness and long-term outcome,  and determine the incidence of “nonbenign” behavior. MATERIALS AND METHODS: In this retrospective study, 60 histologically verified pancreatic NETs incidentally detected with contrast-enhanced MDCT were included. Various MDCT features such as size, morphology, enhancement, and presence of calcifications were evaluated and  were correlated with tumor biology on histopathology. The sensitivity, specificity, predictive values, and accuracy were calculated for MDCT features in predicting nonbenign biology and risk of recurrence. RESULTS: A total of 32 of 60 (53%) NETs were nonbenign: most were large (mean, 29.1 mm) with a solid or complex pattern. NET size of 3 cm or larger yielded a positive predictive value of 61% for nonbenign tumors and 100% when calcification was present. In 12 patients with recurrence, 92% of NETs were nonbenign. The presence of calcification, local invasion, main pancreatic duct dilatation, vascular invasion, and lymph node enlargement along with angioinvasion and a Ki-67 index greater than 2% on histology were associated with a nonbenign diagnosis and a higher risk of recurrence. CONCLUSION: Approximately 50% of incidental NETs show  uncertain or malignant behavior. Solid tumors 3 cm or larger are commonly nonbenign; however, about 30% of tumors smaller than that size cutoff can be malignant. Nonbenign tumors and those with invasive features on MDCT have a higher incidence of recurrence.

 

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[98]

TÍTULO / TITLE:  - Comparative effectiveness of 18F-FDG PET/CT versus whole-body MRI for detection of malignant peripheral nerve sheath tumors in neurofibromatosis type 1.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Clin Nucl Med. 2013 Jan;38(1):e19-25. doi: 10.1097/RLU.0b013e318266ce84.

            ●● Enlace al texto completo (gratuito o de pago) 1097/RLU.0b013e318266ce84

AUTORES / AUTHORS:  - Derlin T; Tornquist K; Munster S; Apostolova I; Hagel C; Friedrich RE; Wedegartner U; Mautner VF

INSTITUCIÓN / INSTITUTION:  - Department of Diagnostic and Interventional Radiology, University Medical Center  Hamburg-Eppendorf, Hamburg, Germany. t.derlin@uke.uni-hamburg.de

RESUMEN / SUMMARY:  - PURPOSE: The aim of this study was to compare the diagnostic performance of (1)(8)F-fluorodeoxyglucose (FDG) PET/CT and whole-body MRI for the detection of malignant peripheral nerve sheath tumors (MPNSTs) in patients with neurofibromatosis type 1, and to evaluate a panel of imaging-based criteria serving that purpose. PATIENTS AND METHODS: Thirty-one patients were examined by  whole-body MRI and (1)(8)F-FDG PET/CT. A panel of imaging-based criteria including tumor region, size, shape, margin definition, contrast enhancement, heterogeneity before and after contrast, intratumoral lobulation, target sign, and mean and maximum standardized uptake values (SUVs) were evaluated. A SUVmax cut-off value of 3.5 was used for lesion analysis. Histopathologic evaluation and/or clinical follow-up served as the reference standard. RESULTS: (1)(8)F-FDG  PET/CT had a sensitivity of 100%, whereas MRI had a sensitivity of 66.7%. On PET/CT, tumor size (P<0.005), SUVmax (P<0.0001), SUVmean (P<0.0001), and tracer uptake heterogeneity (P=0.002) were significantly associated with MPNSTs. On MRI, intratumoral lobulation (P<0.02), ill-defined margins (P=0.007), and irregular enhancement on T1-weighted imaging (P<0.001) were significantly associated with MPNSTs. CONCLUSIONS: Both PET/CT and whole-body MRI may distinguish benign and malignant PNSTs, but PET/CT has higher sensitivity for that purpose. Imaging-based criteria for identification of MPNSTs on both modalities were identified. False-positive results, requiring biopsy or clinical follow-up, may be reduced by using a combination of MRI and PET derived markers, but only at the price of reduced sensitivity.

 

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[99]

TÍTULO / TITLE:  - Primary cardiac pheochromocytoma involving both right and left atria.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Ann Thorac Surg. 2013 Jan;95(1):337-40. doi: 10.1016/j.athoracsur.2012.05.084. Epub 2012 Dec 25.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.athoracsur.2012.05.084

AUTORES / AUTHORS:  - Liu X; Miao Q; Zhang H; Zhang C; Cao L; Ma G; Zeng Z

INSTITUCIÓN / INSTITUTION:  - Department of Cardiac Surgery, Peking Union Medical College Hospital, Beijing, China PR.

RESUMEN / SUMMARY:  - Pheochromocytomas are catecholamine-producing paragangliomas derived from the neural crest. Primary cardiac pheochromocytoma is extremely rare. We describe a case of cardiac pheochromocytoma involving both right and left atria in a middle-aged woman. Metaiodobenzylguanidine (MIBG) scintiscan results were normal, but results from an octreotide scan were abnormal. The tumor was successfully resected using cardiopulmonary bypass (CPB).

 

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[100]

TÍTULO / TITLE:  - Appendix Carcinoids in Childhood: Long-Term Experience at a Single Institution in Western Canada and Systematic Review on Topic.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Pediatr Int. 2013 Jan 2. doi: 10.1111/ped.12047.

            ●● Enlace al texto completo (gratuito o de pago) 1111/ped.12047

AUTORES / AUTHORS:  - Kulkarni KP; Sergi C

INSTITUCIÓN / INSTITUTION:  - Department of Pediatric Oncology, Stollery Children’s Hospital, University of Alberta Hospital, Edmonton, AB, Canada.

RESUMEN / SUMMARY:  - PURPOSE: The objective is to describe our management experience of children with  appendix carcinoids (AC). METHOD: We first performed a systematic review of the literature pertaining to appendix carcinoids in the pediatric population. PubMed, EMBBASE and SCOPUS and most significant Congress proceedings were reviewed. In addition we retrieved the charts and files at our Stollery Children’s Hospital, University of Alberta, Edmonton, Canada. We compared our data with the scientific literature review. RESULTS: Between January 2000 and October 2010, seven patients had AC at our institution. Mean age at diagnosis was 15 years while the male:female ratio was 3:4. Five out of seven patients presented with acute appendicitis. Size ranged up to 1.4 cm. In one case only lymph node metastases were observed. No distant metastases were seen in any patient. Carcinoid was low  grade in all seven children. One patient suffered from FAP. At a mean follow-up of 53.4 months, the outcome was excellent with all patients alive without disease. In the systematic review, we identified 206 studies referring to AC in children. Most series on childhood AC were small single center series with 2-19 patients. CONCLUSIONS: To the best of our knowledge older age (>10 years) at diagnosis, female preponderance, and tumor diameter <1.5 cm were the salient observations in our study. Surgical resection at an early stage allows for excellent prognosis without the need for any additional surgical or adjuvant treatment. Genetic counseling may be appropriate in view of the possible association with colonic adenocarcinoma.

 

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[101]

TÍTULO / TITLE:  - Transformation to “high grade” neuroendocrine carcinoma as an acquired drug resistance mechanism in EGFR-mutant lung adenocarcinoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Lung Cancer. 2013 Jan 9. pii: S0169-5002(12)00712-X. doi: 10.1016/j.lungcan.2012.12.019.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.lungcan.2012.12.019

AUTORES / AUTHORS:  - Popat S; Wotherspoon A; Nutting CM; Gonzalez D; Nicholson AG; O’Brien M

INSTITUCIÓN / INSTITUTION:  - Royal Marsden Hospital NHS Foundation Trust, London, and Surrey, UK; National Heart and Lung Institute, Imperial College London, UK. Electronic address: sanjay.popat@rmh.nhs.uk.

RESUMEN / SUMMARY:  - Several different acquired resistance mechanisms of EGFR mutant lung adenocarcinoma to EGFR-tyrosine kinase inhibitor (TKI) therapy have been described, most recently transformation to small cell lung carcinoma (SCLC). We describe the case of a 46-year-old female with relapsed EGFR exon 19 deletion lung adenocarcinoma treated with erlotinib, and on resistance, cisplatin-pemetrexed. Liver rebiopsy identified an afatinib-resistant combined SCLC and non-small cell carcinoma with neuroendocrine morphology, retaining the EGFR exon 19 deletion. This case highlights acquired EGFR-TKI resistance through  transformation to the high-grade neuroendocrine carcinoma spectrum and that that  such transformation may not be evident at time of progression on TKI therapy.

 

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[102]

TÍTULO / TITLE:  - MEASUREMENT OF CALCITONIN AND CGRP MESSENGER RIBONUCLEIC ACID REFINES THE MANAGEMENT OF PATIENTS WITH MEDULLARY THYROID CANCER AND MAY REPLACE CALCITONIN STIMULATION TEST.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Thyroid. 2012 Dec 23.

            ●● Enlace al texto completo (gratuito o de pago) 1089/thy.2012.0361

AUTORES / AUTHORS:  - Camacho CP; Lindsey SC; Melo MC; Yang JH; Germano-Neto F; Valente FD; Lima TR; Biscolla RP; Vieira JG; Cerutti J; Dias-da-Silva MR; Maciel RM

INSTITUCIÓN / INSTITUTION:  - Universidade Federal de Sao Paulo, Medicine, Sao Paulo, Sao Paulo, Brazil; cpcamacho@unifesp.br.

RESUMEN / SUMMARY:  - Background: Serum calcitonin is the main tumor marker for medullary thyroid cancer (MTC), but it has certain limitations. Various sCT assays may present an important intra-assay and interassay variation and may yield different and sometimes conflicting results. Pentagastrin or calcium-stimulated calcitonin test may be desirable in some situations. Alternatively, or in the absence of the stimulation test, mRNA detection offers the advantages of being more comfortable  and less invasive, being a one-step blood withdrawal, and having no side effects. The objective of this study was to 1) investigate the applicability of measuring  CALCA gene transcripts (CT-CALCA and CGRP-CALCA) in patients with MTC and in relatives diagnosed with a RET mutation and 2) test mRNA as an alternative diagnostic tool for the calcitonin stimulation test. Methods: Twenty-three healthy controls and twenty-six individuals evaluated for MTC were selected, including patients with sporadic or hereditary MTC and RET mutation-carrying relatives. For molecular analysis, RNA was extracted from peripheral blood, followed by cDNA synthesis using 3.5 mug of total RNA. RT-qPCR reactions were performed with SYBR Green, 200 nM of each primer for the two specific mRNA targets (CT-CALCA or CGRP-CALCA) and normalized with the ribosomal protein S8 as  reference gene. Results: We detected CALCA transcripts in the blood samples and observed a positive correlation between them (r: 0.946 and p <0.0001). Both mRNAs also correlated with sCT (CT-CALCA - r: 0.713, p: <0.0001, and CGRP-CALCA - r: 0.714, p: <0.0001). The relative expression of CT-CALCA and CGRP-CALCA presented  higher clinical sensitivity (86.67 and 100, respectively), specificity (97.06 and 97.06), positive predictive value (92.86 and 93.75) and negative predictive value (94.29 and 100), than did sCT (73.33, 82.35, 64.71 and 87.50). In addition, the CALCA transcript measurement mirrors the response to the pentagastrin test. Conclusion: We demonstrated that the measurement of CALCA gene transcripts in the bloodstream is feasible and may refine the management of patients with MTC and RET mutation-carrying relatives. We propose considering the application of this diagnostic tool as an alternative to the calcitonin stimulation test.

 

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[103]

TÍTULO / TITLE:  - A large presacral tailgut cyst with a carcinoid tumor in a male: report of a case.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Surg Today. 2013 Jan 10.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s00595-012-0482-4

AUTORES / AUTHORS:  - Charalampakis V; Stamatiou D; Christodoulakis M; Kafousi M; Chryssou E; de Bree E; Melissas J

INSTITUCIÓN / INSTITUTION:  - Department of Surgical Oncology, Heraklion University Hospital, P.O. Box 1352, 71110, Heraklion, Greece, vcharalambakis@gmail.com.

RESUMEN / SUMMARY:  - Tailgut cysts are developmental hamartomas found in the presacral space. They are usually detected incidentally during physical examinations or imaging studies. However, they may cause symptoms due to compression of nearby organs. Due to their potential malignant transformation, surgical resection is warranted, while  routine biopsy is considered controversial because of the concern about infection of the tailgut cyst and needle-track implantation of malignant cells. The co-existence of a carcinoid in a tailgut cyst is extremely rare. Only 16 cases have been reported previously, the vast majority of which were found in females.  We herein present the case of a carcinoid in a tailgut cyst found in a male patient, discuss the potential pathogenesis of tailgut carcinoids, and underline  the fact that their previous consideration of the condition as a female-restricted entity should be rejected.

 

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[104]

TÍTULO / TITLE:  - Merkel cell carcinoma with sarcomatous differentiation: is it a poor prognostic factor?

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Int J Dermatol. 2013 Jan;52(1):93-5. doi: 10.1111/j.1365-4632.2012.05675.x.

            ●● Enlace al texto completo (gratuito o de pago) 1111/j.1365-4632.2012.05675.x

AUTORES / AUTHORS:  - Gomez-Moyano E; Vera-Casano A; Sanz-Trelles A; Martinez L

INSTITUCIÓN / INSTITUTION:  - Departments of Dermatology Pathology, Hospital Carlos Haya, Malaga, España.

RESUMEN / SUMMARY:  - Background Poor prognostic factors in Merkel cell carcinoma include male sex, advanced stage at diagnosis, large tumor size (>5 mm), diffuse growth pattern, heavy lymphocytic infiltrate, and high mitotic rate. To date only six cases of Merkel cell carcinoma with sarcomatous or pseudosarcomatous differentiation and poor prognosis have been documented. Methods We present a new case of Merkel cell carcinoma with sarcomatous differentiation. Results The immunohistochemical staining patterns reflected the morphologic differentiation of the epithelial and sarcomatous pattern. After two months of follow-up, there were no signs of local  recurrence or metastases. Conclusion In all cases of merkelomas with sarcomatous  differentiation described to date, lymph node metastases have been found, except  in the presented case. However, larger series of cases will be required to determine if sarcomatous differentiation represents another negative prognostic factor.

 

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[105]

TÍTULO / TITLE:  - Cystic pancreatic neuroendocrine tumors: endoscopic ultrasound and fine-needle aspiration characteristics.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endoscopy. 2013 Jan 7.

            ●● Enlace al texto completo (gratuito o de pago) 1055/s-0032-1325990

AUTORES / AUTHORS:  - Yoon WJ; Daglilar ES; Pitman MB; Brugge WR

INSTITUCIÓN / INSTITUTION:  - Gastrointestinal Unit, Massachusetts General Hospital, Boston, Massachusetts, USA.

RESUMEN / SUMMARY:  - Background and study aims: Limited data are available on the endoscopic ultrasound (EUS) and fine-needle aspiration (FNA) characteristics of cystic pancreatic neuroendocrine tumors (CPanNets). The aims of this study were to describe the EUS and FNA characteristics of pathologically confirmed CPanNets and to compare these characteristics with mucinous cysts from matched patients.Patients and methods: From an EUS - FNA database (between 1999 and 2011), 19 patients with a pathologically confirmed CPanNet were identified. Patient characteristics, cyst fluid carcinoembryonic antigen (CEA) levels, pathology, and EUS findings were analyzed. For comparison, age- and sex-matched patients with mucinous cysts were randomly chosen from the same database.Results: Of the 19 patients, two had multiple endocrine neoplasia type 1 and two had metastases. The median diameter of the lesions was 24 mm. EUS revealed unilocular lesions in 7 patients, thinly septated lesions with thin walls in 1, and mixed solid-cystic lesions in 11. EUS - FNA cytology confirmed neoplasm in 12 of the 19 patients (63.2 %). The median cyst fluid CEA level (n = 15) was 1.1 ng/mL (range  0.3 - 500 ng/mL). Compared with matched patients with mucinous cysts, the median  cyst fluid CEA was lower (1.1 ng/mL vs. 400 ng/mL), thick walls were more common  (66.7 % vs. 13.3 %), and diagnostic cytology was more likely (73.3 % vs. 20.0 %).Conclusions: Analysis of EUS and FNA results showed that the cyst fluid from CPanNets had a lower CEA concentration, a higher frequency of thick walls on EUS, and higher diagnostic cytology compared with mucinous cysts. These findings may aid in the diagnosis of CPanNets.

 

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[106]

- CASTELLANO -

TÍTULO / TITLE:Neuromas mucosos de localizacion laringea en una neoplasia endocrina multiple 2B.

TÍTULO / TITLE:  - Multiple mucosal neuromas in the larynx as part of a multiple endocrine neoplasia type 2B.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Acta Otorrinolaringol Esp. 2012 Dec 3. pii: S0001-6519(12)00225-7. doi: 10.1016/j.otorri.2012.09.008.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.otorri.2012.09.008

AUTORES / AUTHORS:  - Soroa-Ruiz F; Lara-Sanchez H; Ramirez Anguiano J; Cordova-Ramon JC

INSTITUCIÓN / INSTITUTION:  - Servicio de Otorrinolaringologia y Cirugia de Cabeza y Cuello, Instituto Nacional de Ciencias Medicas y Nutricion <<Salvador Zubiran>>, Mexico, DF, Mexico. Electronic address: pacosoroa@yahoo.com.

 

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[107]

TÍTULO / TITLE:  - The surgical management of neuroendocrine tumour hepatic metastases.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Eur J Surg Oncol. 2013 Jan 2. pii: S0748-7983(12)01337-6. doi: 10.1016/j.ejso.2012.12.001.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.ejso.2012.12.001

AUTORES / AUTHORS:  - Pathak S; Dash I; Taylor MR; Poston GJ

INSTITUCIÓN / INSTITUTION:  - Royal United Hospital Bath NHS Trust, Combe Park, Bath BA1 3NG, United Kingdom.

RESUMEN / SUMMARY:  - Although rare in incidence, patients with neuroendocrine tumours (NET) live for many years and so have a high prevalence, and NETs frequently metastasise to the  liver (NLM). Numerous treatment options have been implemented both for cure, and  to implement disease control. Surgical treatment consists of curative resection,  palliative cytoreductive resection and transplantation. Complete surgical resection is possible only in a subset of people with NLMs due to various factors. Ablative therapies may also be used, either as an adjunct to surgery or  as a primary treatment. The purpose of the article is to summarise surgical treatment strategies in the management of patients with hepatic neuroendocrine metastases.

 

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[108]

TÍTULO / TITLE:  - Cabozantinib approved for advanced medullary thyroid cancer.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Am J Health Syst Pharm. 2013 Jan 1;70(2):88. doi: 10.2146/news130005.

            ●● Enlace al texto completo (gratuito o de pago) 2146/news130005

AUTORES / AUTHORS:  - Traynor K

 

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[109]

TÍTULO / TITLE:  - Intraductal Polypoid Lipid-Rich Neuroendocrine Tumor of the Pancreas with Entrapped Ductules: Case Report and Review of the Literature.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endocr Pathol. 2013 Jan 13.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s12022-012-9231-x

AUTORES / AUTHORS:  - Hechtman JF; Franssen B; Labow DM; Gordon RE; Dimaio CJ; Wilck EJ; Carrasco-Avino G; Zhu H

INSTITUCIÓN / INSTITUTION:  - Department of Pathology, Mount Sinai School of Medicine, One Gustave L Levy Place, 1194, New York, NY, 10029, USA, jaclyn.hechtman@mountsinai.org.

RESUMEN / SUMMARY:  - Pancreatic neuroendocrine tumors of the main pancreatic duct are rare and usually small due to symptoms of pancreatic duct obstruction. We present a case of a large (3 cm), well-differentiated (G1) lipid-rich polypoid neuroendocrine tumor of the pancreas completely occluding the main pancreatic duct with non-neoplastic-entrapped ductules and CK19 positivity. Clinical, radiological, gross, microscopic, immunohistochemical, and ultrastructural findings are discussed. The literature pertaining to the unique features of this case is reviewed including clinical and pathologic pitfalls and the possible etiologic and prognostic significance of these findings.

 

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[110]

TÍTULO / TITLE:  - Metastatic Medullary Carcinoma of Thyroid Presenting as a Dural-Based Mass: Case  Report and Review of Literature.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endocr Pathol. 2013 Jan 25.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s12022-013-9233-3

AUTORES / AUTHORS:  - Rishi A; Savona S; Black K; Schulder M; Li JY

INSTITUCIÓN / INSTITUTION:  - Department of Pathology and Laboratory Medicine, North Shore-Long Island Jewish Health System, Hofstra North Shore-LIJ School of Medicine, 6 Ohio Drive, Suite 202, Lake Success, NY, 11042, USA.

RESUMEN / SUMMARY:  - Dural metastasis from medullary thyroid carcinoma (MTC) is not well established in English literature. We present the case report of MTC with unusual clinical presentation as a dural-based mass in a 39-year-old male with no family history of multiple endocrine neoplasia syndrome. Magnetic resonance imaging showed an extra-axial dural-based mass in right frontal lobe with calvarium and soft tissue extension to the right superior orbit. Histopathology showed MTC with variegated  morphology and various patterns. Thyroid mass and widespread metastases from medullary thyroid carcinoma were subsequently identified.

 

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[111]

TÍTULO / TITLE:  - (18)F-DOPA PET/CT revealed synchronous neuroendocrine tumors in two sisters with  MEN2A syndrome.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endocrine. 2012 Dec 11.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s12020-012-9854-1

AUTORES / AUTHORS:  - Treglia G; Castaldi P; Rufini V

INSTITUCIÓN / INSTITUTION:  - Institute of Nuclear Medicine, Catholic University of the Sacred Heart, Largo A.  Gemelli 8, 00168, Rome, Italy, giorgiomednuc@libero.it.

 

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[112]

TÍTULO / TITLE:  - Neuroendocrine carcinoma of the urinary bladder: a retrospective study of CT findings.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Abdom Imaging. 2012 Dec 1.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s00261-012-9971-6

AUTORES / AUTHORS:  - Boyer AC; Jafri SZ; Jafri SM; Amin MB

INSTITUCIÓN / INSTITUTION:  - Department of Radiology, Oakland University William Beaumont School of Medicine,  2 Center Tower, 3601 W. Thirteen Mile Road, Royal Oak, MI, 48073, USA, andrewc.boyer@beaumont.edu.

RESUMEN / SUMMARY:  - PURPOSE: The purpose of this study was to evaluate the radiographic features of neuroendocrine carcinoma of the urinary bladder (NECB) on CT and to review the literature regarding carcinogenesis, treatment, and prognosis. METHODS: The presenting CT of patients with pathology-proven NECB were retrospectively reviewed for features including size and appearance of the bladder mass, the presence of hydronephrosis, bladder wall thickening, invasion of perivesical fat, lymph nodes, and distant metastasis. Follow-up imaging and the medical record were reviewed to determine patient treatment and overall survival. RESULTS: Sixteen patients (13 males, 3 females) were diagnosed with NECB with a mean age of 75.5 years (range 48-90). The characteristic CT appearance was a large polypoid bladder mass (average size 4.9 cm). Extension into the perivesical fat,  adjacent organ involvement, and distant metastases were common. CONCLUSION: NECB  is an aggressive primary neoplasm of the bladder that presents on CT as a large bladder mass with local extension into the perivesical fat, involvement of adjacent organs, and metastasis.

 

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[113]

TÍTULO / TITLE:  - Plasma S100beta is not a useful biomarker for tumor burden in neurofibromatosis.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Clin Biochem. 2012 Dec 19. pii: S0009-9120(12)00695-9. doi: 10.1016/j.clinbiochem.2012.12.007.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.clinbiochem.2012.12.007

AUTORES / AUTHORS:  - Smith MJ; Esparza S; Merker VL; Muzikansky A; Bredella MA; Harris GJ; Kassarjian A; Cai W; Walker JA; Mautner VF; Plotkin SR

INSTITUCIÓN / INSTITUTION:  - Department of Neurology, Massachusetts General Hospital, Boston, MA, USA.

RESUMEN / SUMMARY:  - OBJECTIVES: Neurofibromatosis 1 (NF1), NF2, and schwannomatosis are characterized by a predisposition to develop multiple neurofibromas and schwannomas. Currently, there is no blood test to estimate tumor burden in patients with these disorders. We explored whether S100beta would act as a biomarker of tumor burden in NF since S100beta is a classic immunohistochemical marker of astrocytes, oligodendrocytes  and Schwann cells and a small study showed S100beta concentrations correlate with the volume of vestibular schwannomas. DESIGN AND METHODS: We calculated whole-body tumor burden in subjects with NF1, NF2, and schwannomatosis using whole-body MRI (WBMRI) and measured the concentration of S100beta in plasma using ELISA. We used chi-square tests and Spearman rank correlations to test the relationship between S100beta levels and whole-body tumor burden. RESULTS: 127 consecutive patients were enrolled in the study (69 NF1 patients, 28 NF2 patients, and 30 schwannomatosis patients). The median age was 40years, 43% were  male, and median whole-body tumor volume was 26.9mL. There was no relationship between the presence of internal tumors and the presence of detectable S100beta in blood for the overall group or for individual diagnoses (p>0.05 by chi-square  for all comparisons). Similarly, there was no correlation between whole-body tumor volume and S100beta concentration for the overall group or for individual diagnoses (p>0.05 by Spearman for all comparisons). CONCLUSIONS: Plasma S100beta  is not a useful biomarker for tumor burden in the neurofibromatoses. Further work is needed to identify a reliable biomarker of tumor burden in NF patients.

 

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[114]

TÍTULO / TITLE:  - Targeting RET to induce medullary thyroid cancer cell apoptosis: an antagonistic  interplay between PI3K/Akt and p38MAPK/caspase-8 pathways.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Apoptosis. 2013 Jan 18.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s10495-013-0803-0

AUTORES / AUTHORS:  - Mazumdar M; Adhikary A; Chakraborty S; Mukherjee S; Manna A; Saha S; Mohanty S; Dutta A; Bhattacharjee P; Ray P; Chattopadhyay S; Banerjee S; Chakraborty J; Ray AK; Sa G; Das T

INSTITUCIÓN / INSTITUTION:  - Division of Molecular Medicine, Bose Institute, P-1/12, Calcutta Improvement Trust Road, Scheme VII M, Kolkata, West Bengal, 700 054, India.

RESUMEN / SUMMARY:  - Mutations in REarranged during Transfection (RET) receptor tyrosine, followed by  the oncogenic activation of RET kinase is responsible for the development of medullary thyroid carcinoma (MTC) that responds poorly to conventional chemotherapy. Targeting RET, therefore, might be useful in tailoring surveillance of MTC patients. Here we showed that theaflavins, the bioactive components of black tea, successfully induced apoptosis in human MTC cell line, TT, by inversely modulating two molecular pathways: (i) stalling PI3K/Akt/Bad pathway that resulted in mitochondrial transmembrane potential (MTP) loss, cytochrome-c release and activation of the executioner caspases-9 and -3, and (ii) upholding p38MAPK/caspase-8/caspase-3 pathway via inhibition of Ras/Raf/ERK. Over-expression of either constitutively active myristoylated-Akt-cDNA (Myr-Akt-cDNA) or dominant-negative-caspase-8-cDNA (Dn-caspase-8-cDNA) partially  blocked theaflavin-induced apoptosis, while co-transfection of Myr-Akt-cDNA and Dn-caspase-8-cDNA completely eradicated the effect of theaflavins thereby negating the possibility of existence of other pathways. A search for the upstream signaling revealed that theaflavin-induced disruption of lipid raft caused interference in anchorage of RET in lipid raft that in turn stalled phosphorylation of Ras and PI3Kinase. In such anti-survival cellular micro-environment, pro-apoptotic signals were triggered to culminate into programmed death of MTC cell. These findings not only unveil a hitherto unexplained mechanism underlying theaflavin-induced MTC death, but also validate  RET as a promising and potential target for MTC therapy.

 

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[115]

TÍTULO / TITLE:  - Intravitreal bevacizumab for iris tumor metastasized from large cell neuroendocrine carcinoma of lung.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Graefes Arch Clin Exp Ophthalmol. 2012 Dec 19.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s00417-012-2218-y

AUTORES / AUTHORS:  - Yokouchi H; Kitahashi M; Oshitari T; Yamamoto S

INSTITUCIÓN / INSTITUTION:  - Department of Ophthalmology and Visual Science, Chiba University Graduate School  of Medicine, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8670, Japan, yokouchi123@peace.ocn.ne.jp.

 

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[116]

TÍTULO / TITLE:  - Divergent Management Strategies for Typical Versus Atypical Carcinoid Tumors of the Thoracic Cavity.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Am J Clin Oncol. 2013 Jan 24.

            ●● Enlace al texto completo (gratuito o de pago) 1097/COC.0b013e31827a7f6d

AUTORES / AUTHORS:  - Okoye CC; Jablons DM; Jahan TM; Kukreja J; Cardozo S; Yom SS

INSTITUCIÓN / INSTITUTION:  - Departments of *Radiation Oncology daggerSurgery, Division of Adult Cardiothoracic Surgery double daggerMedicine, Division of Medical Oncology, Helen Diller Family Comprehensive Cancer Center, University of California, San Francisco, CA.

RESUMEN / SUMMARY:  - OBJECTIVES:: At our institution, limited surgical techniques are reserved only for suspected typical carcinoids, and nodal dissection and multimodality therapy  are frequently used for atypical carcinoids. We describe the results of these differing management strategies based on initial clinicopathologic characteristics. METHODS:: Retrospective review of patients treated for thoracic  carcinoid from 1995 to 2009. Information was abstracted concerning surgical and nonsurgical treatments, pathology results, and outcomes. Event-free and survival  endpoints were compared. RESULTS:: The median follow-up was 5.0 years (range, 0.5 to 17.4 y). Fifty-two patients underwent resection. The 5-year event-free survival for typical carcinoid patients was 88.2%. Atypical carcinoids had a tendency for nodal involvement (50% vs. 15%) and greater likelihood for disease recurrence, with a 5-year event-free survival of 50%. CONCLUSIONS:: These data support the appropriateness of divergent management strategies for typical versus atypical bronchial carcinoids. We propose the following: (1) nonanatomic resection is acceptable only for peripheral typical carcinoids; (2) extended mediastinal dissection should be limited to central presentations, clinically aggressive, or atypical carcinoids; (3) atypical histology, especially with nodal involvement, is prognostic for recurrence and metastasis; (4) nonsurgical therapies only rarely achieve long-term freedom from disease.

 

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[117]

TÍTULO / TITLE:  - Merkel cell carcinoma of the upper eyelid: presentation and management.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Int J Oral Maxillofac Surg. 2012 Dec 6. pii: S0901-5027(12)00446-8. doi: 10.1016/j.ijom.2012.10.031.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.ijom.2012.10.031

AUTORES / AUTHORS:  - Baccarani A; Pompei B; Pedone A; Brombin A

INSTITUCIÓN / INSTITUTION:  - Division of Plastic Surgery, Azienda Ospedaliero-Universitaria Policlinico di Modena, University of Modena and Reggio Emilia, Italy. Electronic address: alessio.baccarani@unimore.it.

RESUMEN / SUMMARY:  - Merkel cell carcinoma (MCC) is a rare and potentially aggressive neuroendocrine tumour. The authors describe a unique presentation of a 4.5cm wide MCC of the upper lid in a 73-year-old female. After total upper lid resection, immediate reconstruction was achieved by a full-thickness lower-lid transposition flap based on the lower lateral palpebral artery. At the 3 year follow-up the patient  is free from disease and the reconstructive result is satisfactory both functionally and aesthetically.

 

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[118]

TÍTULO / TITLE:  - Elevated CA 19-9 Levels Observed in Association with a Pulmonary Neuroendocrine Tumour and Amyloid.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Intern Med. 2013;52(1):105-9. Epub 2013 Jan 1.

AUTORES / AUTHORS:  - Faruqi S; Gumparthy K; Wahbi Z

INSTITUCIÓN / INSTITUTION:  - Department of Respiratory Medicine, Castle Hill Hospital, UK.

RESUMEN / SUMMARY:  - Investigations completed in a 77-year-old ex-smoker presenting with weight loss showed raised CA 19-9 levels. The findings of chest radiograph, abdominal computed tomography (CT) and gastrointestinal endoscopic examinations were all normal. On follow-up, the patient developed left upper lobe collapse on chest radiograph with increasing CA 19-9 levels. Chest CT findings suggested the presence of a left upper lobe tumour. The results of a biopsy of the left upper lobe nodule seen on bronchoscopy suggested a diagnosis of amyloidosis; however, this was not the only diagnosis. The left upper lobe mass comprised a neuroendocrine tumour with amyloid deposition just beneath the bronchial epithelium and focally between the nests of the tumour cells. We report and discuss this uncommon association and presentation.

 

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[119]

TÍTULO / TITLE:  - Juvenile-onset Hereditary Pheochromocytoma-paraganglioma Syndrome.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Intern Med. 2013;52(2):281-4. Epub 2013 Jan 15.

AUTORES / AUTHORS:  - Sugisawa C; Okada Y; Arao T; Mori H; Nishida K; Isobe K; Takekoshi K; Tanaka Y

INSTITUCIÓN / INSTITUTION:  - The First Department of Internal Medicine, School of Medicine, University of Occupational and Environmental Health, Japan.

RESUMEN / SUMMARY:  - It is insufficient to distinguish benign tumors from malignant pheochromocytoma using histological analyses of resected tissue alone. We experienced an 18-year-old woman who complained of severe headaches in whom hypertension was revealed. She was suspected of having a malignant tumor based on her clinical characteristics, despite showing no evidence of metastatic lesions. The patient was diagnosed with an aggressive form of hereditary pheochromocytoma-paraganglioma syndrome (HPPS) based on immunohistochemical analyses and genetic testing. The present case indicates that conducting genetic  testing, including SDHB mutation analyses, is required to determine the prognosis in patients highly suspected of having HPPS.

 

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[120]

TÍTULO / TITLE:  - Neuroendocrine carcinoma of the pancreas with soft tissue metastasis.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - World J Gastroenterol. 2012 Dec 7;18(45):6682-5. doi: 10.3748/wjg.v18.i45.6682.

            ●● Enlace al texto completo (gratuito o de pago) 3748/wjg.v18.i45.6682

AUTORES / AUTHORS:  - Chen J; Zheng Q; Yang Z; Huang XY; Yuan Z; Tang J

INSTITUCIÓN / INSTITUTION:  - Department of Surgery, Shanghai 6th People’s Hospital Affiliated to Shanghai Jiaotong University, Shanghai 200233, China.

RESUMEN / SUMMARY:  - Neuroendocrine carcinoma (NEC) of the pancreas is rare. We report the case of a 34-year-old man with pancreatic NEC with soft tissue metastasis. The patient presented with right upper abdominal discomfort. Computed tomography revealed a low-density heterogeneous mass in the tail and body of the pancreas that encroached on the greater curvature of the stomach and spleen. We performed exploratory laparotomy and total pancreatectomy with splenectomy and total gastrectomy. Histopathological analysis showed spindle-shaped cells with scanty cytoplasm and hyperchromatic nuclei, confirming a primary pancreatic NEC. One month after the surgery, the patient experienced leg swelling. Positron emission  tomography-computed tomography revealed high uptake of fludeoxyglucose in the left leg, and the leg was amputated. Histopathological analysis confirmed metastasis of pancreatic NEC. The patient was followed up and received chemotherapy (etoposide and cisplatin). One month after amputation, the level of  tumor marker neuron-specific enolase was 142.70 mug/L and computed tomography scan revealed an aggravated metastatic lesion. The patient suffered from unbearable pain and we treated him with odynolysis. Four months postoperatively,  the patient died of respiratory failure.

 

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[121]

TÍTULO / TITLE:  - Gastric Neuroendocrine Tumors in Our Institutions According to the WHO 2010 Classification.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Int Surg. 2012 Oct;97(4):335-9. doi: 10.9738/CC134.1.

            ●● Enlace al texto completo (gratuito o de pago) 9738/CC134.1

AUTORES / AUTHORS:  - Endo S; Dousei T; Yoshikawa Y; Hatanaka N; Taniyama K; Yamauchi A; Kamiike W; Nishijima J

INSTITUCIÓN / INSTITUTION:  - 1 Department of Surgery, Higashiosaka City General Hospital, Higashiosaka-shi, Osaka, Japan.

RESUMEN / SUMMARY:  - Abstract In 2010, World Health Organization classified gastric neuroendocrine tumor (NET) as follows: NET grade (G) 1, NET G2, neuroendocrine carcinoma (NEC).  We reviewed 22 gastric NETs that were encountered in our institutions. Nine, 6, and 4 were NET G1, G2, and NEC, respectively. We also encountered 3 NET G3. NET G1 was treated with observation in 2 patients, endoscopic mucosal resection (EMR) in 3, and gastrectomy in 4 patients. No recurrence was experienced during a median of 53 months of follow-up. All NET G2 was treated with gastrectomy. No patient experienced recurrence during a median of 25 months of follow-up. NET G3  was treated with gastrectomy. One patient died of liver metastasis 52 months after gastrectomy. For NEC, gastrectomy was performed in 3 cases and no patients  died of tumor-related death. We conclude that the prognoses of NET G1 and G2 were good. We also experienced long-term survivors of NEC. An accumulation of more patients is needed for further investigation.

 

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[122]

TÍTULO / TITLE:  - Usual and Unusual Neuroendocrine Tumor Metastases on 68Ga-DOTANOC PET/CT: A Pictorial Review.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Clin Nucl Med. 2012 Dec 10.

            ●● Enlace al texto completo (gratuito o de pago) 1097/RLU.0b013e318252d2c3

AUTORES / AUTHORS:  - Naswa N; Sharma P; Kumar R; Malhotra A; Bal C

INSTITUCIÓN / INSTITUTION:  - From the Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India.

RESUMEN / SUMMARY:  - ABSTRACT: Neuroendocrine tumors (NETs) are slow-growing indolent tumors that often present with metastatic disease at the outset. They commonly metastasize to lymph nodes, liver, bone, and lungs. However, metastasis to other rare sites may  occur. It is important to have clear knowledge of unusual NET metastatic sites because their presence may lead to a more directed investigation. Also, it will be helpful in ruling out incidental second malignancies that might be encountered. The objective of this pictorial article was to provide an illustrative tutorial showing the clinical utility of Ga-labeled somatostatin analog [1, 4, 7, 10-tetraazacyclododecane-1, 4, 7, 10-tetraacetic acid]-1-NaI3-octreotide (Ga-DOTANOC) PET/CT for imaging usual and unusual metastatic sites in patients with NETs.

 

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[123]

TÍTULO / TITLE:  - Combined Large Cell Neuroendocrine Carcinoma and Adenocarcinoma of the Gallbladder.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endocr Pathol. 2012 Dec 15.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s12022-012-9229-4

AUTORES / AUTHORS:  - Al-Brahim N; Albannai R

INSTITUCIÓN / INSTITUTION:  - Department of Pathology, Farwaniya Hospital, Kuwait City, Kuwait, nabeel.albrahim@gmail.com.

 

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[124]

TÍTULO / TITLE:  - Recurrent Acute-Onset Cushing’s Syndrome 6 Years after Removal of a Thymic Neuroendocrine Carcinoma: From Ectopic ACTH to CRH.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endocr Pathol. 2012 Dec 13.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s12022-012-9228-5

AUTORES / AUTHORS:  - Schalin-Jantti C; Asa SL; Arola J; Sane T

INSTITUCIÓN / INSTITUTION:  - Division of Endocrinology, Department of Medicine, Helsinki University Central Hospital, P.O. Box 340, 00290, Helsinki, Finland, camilla.schalin-jantti@hus.fi.

RESUMEN / SUMMARY:  - We describe a rare case of ectopic Cushing’s syndrome that recurred 6 years after resection of a thymic neuroendocrine carcinoma. We discuss reasons for the differing clinical presentations, management, hormone profiles, as well as immunopathology. A 41-year-old male developed acute-onset Cushing’s syndrome. Clinical presentation and laboratory results were compatible with ectopic adrenocorticotropin hormone (ACTH) production. Computerized tomography (CT) showed a 3.6 cm thymic tumor which was successfully resected. Plasma ACTH (P-ACTH) normalized the first postoperative day. Histopathology demonstrated a well-differentiated neuroendocrine carcinoma with diffuse positivity for ACTH and focal corticotropin-releasing hormone (CRH) reactivity in a few scattered cells.  The patient was in remission for 6 years. He then again presented with acute-onset Cushing’s syndrome. Fluorine-labeled dihydroxyphenylalanine ((18)F-DOPA) PET/CT showed local uptake in the mediastinum and he underwent repeat resection. However, P-ACTH remained increased (613 ng/l) and 24-h urinary  cortisol was 36,720 nmol, suggesting incomplete tumor removal or metastatic spread. Metyrapone treatment was initiated but then withdrawn because the patient spontaneously recovered and cortisol metabolism gradually normalized within 3 weeks. Histopathology demonstrated a recurrent neuroendocrine carcinoma with the  same features as the previous lesion but this time CRH was strongly positive in more numerous cells. Normalization of P-ACTH after primary surgery was compatible with ectopic ACTH production. However, the delayed fall in P-ACTH and serum cortisol is compatible with ectopic CRH production and stimulation of pituitary ACTH secretion, which gradually resolved. Although ectopic CRH production is very rare, the unusual dynamics illustrated here should raise the possibility of CRH production by a neuroendocrine tumor.

 

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[125]

TÍTULO / TITLE:  - MULTIDISCIPLINARY MANAGEMENT IN MERKEL CELL CARCINOMA.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Dermatolog Treat. 2012 Dec 10.

            ●● Enlace al texto completo (gratuito o de pago) 3109/09546634.2012.756969

AUTORES / AUTHORS:  - Matkowski R; Lata E; Zietek M; Halon A; Forgacz J; Szynglarewicz B

INSTITUCIÓN / INSTITUTION:  - Department of Oncology and Division of Oncological Surgery, Wroclaw Medical University , Plac Hirszfelda 12, 53 413 Wroclaw , Poland.

RESUMEN / SUMMARY:  - Abstract Herein, we describe our experience with the treatment of Merkel cell carcinoma (MCC) and review the literature regarding MCC treatment regimens. Nine  patients underwent treatment due to stage I, II or III MCC. The median follow-up  was 39 months. In 5 cases, tumors were excised with skin margins of >2 cm, and skin margins were <2 cm in 4 patients. Local adjuvant radiotherapy (RT) was given to 4 patients, while 3 patients underwent local lymphadenectomy (LAD). Local recurrence occurred in 4 patients who did not undergo RT (among them 3 with excision margins <2 cm) after a mean time of 9 months. Despite re-treatment 2 of  those patients developed metastases. Recurrence-free survival after primary therapy was achieved: (a) in 3 patients with stage I and II MCC treated surgically with excision margins <2 cm combined with radiotherapy or wide excision >2 cm alone (b) in 2 patients with stage III MCC treated with wide excision and LAD combined with local and regional RT. A review of the literature  supports the following recommendations: (a) Excision with adequate margins combined with RT. (b) LAD with regional radiotherapy in cases of lymph node involvement. (c) SLNB in patients without clinically suspicious lymph nodes.

 

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[126]

TÍTULO / TITLE:  - Pancreatic neuroendocrine tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Dis Mon. 2013 Jan;59(1):5-19. doi: 10.1016/j.disamonth.2012.10.002.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.disamonth.2012.10.002

AUTORES / AUTHORS:  - Muniraj T; Vignesh S; Shetty S; Thiruvengadam S; Aslanian HR

 

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[127]

TÍTULO / TITLE:  - Vandetanib and the management of advanced medullary thyroid cancer.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Curr Opin Oncol. 2013 Jan;25(1):39-43. doi: 10.1097/CCO.0b013e32835a42b9.

            ●● Enlace al texto completo (gratuito o de pago) 1097/CCO.0b013e32835a42b9

AUTORES / AUTHORS:  - Campbell MJ; Seib CD; Gosnell J

INSTITUCIÓN / INSTITUTION:  - Department of Endocrine Surgery, University of California, San Francisco, San Francisco, California, USA.

RESUMEN / SUMMARY:  - PURPOSE OF REVIEW: Vandetanib is a small molecule tyrosine kinase inhibitor that  has been recently approved as an ‘orphan drug’ for the treatment of patients with unresectable, locally advanced, or metastatic medullary thyroid cancer (MTC). RECENT FINDINGS: MTC is a neuroendocrine malignancy frequently associated with mutations to the RET proto-oncogene. Vandetanib selectively targets RET, vascular endothelial growth factor receptor-2, and epidermal growth factor receptor dependent signaling. Vandetanib has been shown to improve progression-free survival in patients with advanced MTC. In general, vandetanib is well tolerated, but QTc prolongation remains a potential concern demanding careful patient selection and monitoring. SUMMARY: Vandetanib has emerged as one of the more promising small molecule tyrosinse kinase inhibitors, providing durable rates of  disease stabilization, with an acceptable adverse event profile in patients with  advanced MTC.

 

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[128]

TÍTULO / TITLE:  - Clinical presentation, recurrence, and survival in patients with neuroendocrine tumors: results from a prospective institutional database.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endocr Relat Cancer. 2013 Jan 14.

            ●● Enlace al texto completo (gratuito o de pago) 1530/ERC-12-0340

AUTORES / AUTHORS:  - Ter-Minassian M; Chan JA; Hooshmand SM; Brais LK; Daskalova A; Heafield R; Buchanan L; Qian ZR; Fuchs CS; Lin X; Christiani DC; Kulke MH

INSTITUCIÓN / INSTITUTION:  - M Ter-Minassian, Medical Oncology, Dana-Farber Cancer Institute, Boston, 02115, United States.

RESUMEN / SUMMARY:  - The rarity of neuroendocrine tumors (NET) has contributed to a paucity of large epidemiologic studies of patients with this condition. We characterized presenting symptoms and clinical outcomes in a prospective database of over 900 patients with NET. We used data from patient questionnaires and the medical record to characterize presenting symptoms, disease-free survival (DFS) and overall survival (OS). The majority of patients in this database had gastroenteropancreatic NET. The median duration of patient-reported symptoms prior to diagnosis was 3.4 months; 19.5% reported durations from 1 to 5 years, 2.5% from 5 to 10 years and 2% greater than 10 years. The median DFS among patients with resected small bowel NET or pancreatic NET was 5.8 yrs and 4.1 years, respectively. After correcting for left truncation bias, the median OS was 7.9 yrs for advanced small bowel NET and 3.9 yrs for advanced pancreatic NET. Chromogranin A (CGA) above twice the upper limit of normal was associated with shorter survival times (HR 2.8 (1.9, 4.0) p<0.001) patients with metastatic disease, regardless of tumor subtype. Our data suggest that while most NET patients are diagnosed soon after symptom onset, prolonged symptom duration prior to diagnosis is a prominent feature of this disease. Though limited to observations from a large referral center, our observations confirm the prognostic value of CGA, and suggest that median survival durations may be shorter than reported in other institutional databases.

 

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[129]

TÍTULO / TITLE:  - Genetic analysis in young patients with sporadic pituitary macroadenomas:Beside AIP don’t forget MEN1 genetic analysis.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Eur J Endocrinol. 2013 Jan 15.

            ●● Enlace al texto completo (gratuito o de pago) 1530/EJE-12-0763

AUTORES / AUTHORS:  - Cuny T; Pertuit M; Sahnoun-Fathallah M; Daly AF; Occhi G; Odou MF; Tabarin A; Nunes ML; Delemer B; Rohmer V; Desailloud R; Kerlan V; Chabre O; Sadoul JL; Cogne M; Caron P; Cortet C; Lienhardt-Roussie A; Raingeard I; Guedj AM; Brue T; Beckers A; Weryha G; Enjalbert A; Barlier A

INSTITUCIÓN / INSTITUTION:  - T Cuny, Endocrinology, University Hospital of Nancy Brabois, Vandoeuvre-Les-Nancy, France.

RESUMEN / SUMMARY:  - CONTEXT: germline mutations in the AIP gene have been identified in young patients (age </= 30 years old) with sporadic pituitary macroadenomas. Otherwise, there are few data concerning the prevalence of MEN1 mutations in such population. OBJECTIVE: We assessed the prevalence of both AIP and MEN1 genetic abnormalities (mutations and large gene deletions) in young patients (age </= 30  years old) diagnosed with sporadic and isolated macroadenoma, without hypercalcemia and/or MEN1-associated lesions. DESIGN: The entire coding sequences of AIP and MEN1 were screened for mutations. In cases of negative sequencing screening, multiplex ligation-dependent probe amplification was performed for the detection of large genetic deletions. PATIENTS AND SETTINGS: 174 patients from Endocrinology Departments of 15 French University Hospital Centers were eligible  for this study. RESULTS: 21/174(12%) patients had AIP (n=15, 8.6%) or MEN1 (n=6,  3.4%) mutations. In pediatric patients (age </= 18 years old), AIP/MEN1 mutation  frequency reached nearly 22% (n=10/46). AIPmut and MEN1mut were respectively identified in 8/79 (10.1%) and 1/79 (1.2%) somatotropinoma patients; they each accounted for 4/74 (5.4%) prolactinoma patients with mutations. Half of patients  (n=3/6) with gigantism displayed mutations in AIP. Interestingly, 4/12 (33%) patients with non-secreting adenomas bore either AIP or MEN1 mutations, whereas none of the 8 corticotroph-adenomas and a single thyrotropinoma case had mutations. No large gene deletions were observed in sequencing-negative patients. CONCLUSION: mutations in MEN1 can be of significance in young patients with sporadic isolated pituitary macroadenomas, particularly prolactinomas, and together with AIP, we suggest genetic analysis of MEN1 in such population.

 

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[130]

TÍTULO / TITLE:  - Everolimus Plus Octreotide Long-Acting Repeatable in Patients With Colorectal Neuroendocrine Tumors: A Subgroup Analysis of the Phase III RADIANT-2 Study.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Oncologist. 2013;18(1):46-53. Epub 2012 Dec 21.

            ●● Enlace al texto completo (gratuito o de pago) 1634/theoncologist.2012-0263

AUTORES / AUTHORS:  - Castellano D; Bajetta E; Panneerselvam A; Saletan S; Kocha W; O’Dorisio T; Anthony LB; Hobday T

INSTITUCIÓN / INSTITUTION:  - Medical Oncology Department, Hospital Universitario 12 de Octubre, Avenida de Cordoba, 28041 Madrid, España. cdanicas@hotmail.com.

RESUMEN / SUMMARY:  - Introduction. The incidence of colorectal neuroendocrine tumors (NETs) is increasing, and patients with this disease have particularly poor prognoses. Treatment options are limited, and survival times have not improved in the past decade. Methods. A post hoc analysis of the efficacy and tolerability of everolimus plus octreotide long-acting repeatable (LAR) was conducted in patients with colorectal NETs enrolled in the phase III RAD001 in Advanced Neuroendocrine  Tumors, Second Trial (RADIANT-2) study. The primary endpoint (progression-free survival [PFS]), secondary endpoints (including objective response rate), and safety were assessed. Results. Patients with colorectal NETs receiving everolimus plus octreotide LAR had a significantly longer median PFS (29.9 months; n = 19) than did those receiving placebo plus octreotide LAR (6.6 months; n = 20). Everolimus plus octreotide LAR treatment also significantly reduced the risk for  disease progression (hazard ratio: 0.34; 95% confidence interval: 0.13-0.89; p =  .011). Although no objective responses were observed, tumor shrinkage was more frequently noted in the everolimus plus octreotide LAR arm than in the placebo plus octreotide LAR arm (67% vs. 37%, respectively). The combination of everolimus plus octreotide LAR was generally well tolerated by patients with colorectal NETs; rash and stomatitis were the most commonly reported adverse events. Conclusions. Everolimus plus octreotide LAR treatment had significant benefits and improved outcomes for patients with advanced colorectal NETs compared with placebo plus octreotide LAR treatment. Results of this exploratory  analysis are consistent with those reported from the RADIANT-2 primary analysis.  These findings support additional investigations of everolimus plus octreotide LAR in patients with colorectal NETs.

 

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[131]

TÍTULO / TITLE:  - Carcinoid Heart Disease: Outcome After Balloon Pulmonary Valvuloplasty.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Can J Cardiol. 2012 Dec 7. pii: S0828-282X(12)01367-0. doi: 10.1016/j.cjca.2012.09.015.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.cjca.2012.09.015

AUTORES / AUTHORS:  - Carrilho-Ferreira P; Silva D; Almeida AG; Infante de Oliveira E; Ferreira C; Miranda L; Rosa R; Canas da Silva P; Bicha Castelo H; Nunes Diogo A

INSTITUCIÓN / INSTITUTION:  - Department of Cardiology, Santa Maria University Hospital, CHLN, Lisbon, Portugal; Lisbon School of Medicine, University of Lisbon, Lisbon, Portugal. Electronic address: pcarrilhoferreira@gmail.com.

RESUMEN / SUMMARY:  - Carcinoid heart disease typically presents with pulmonary stenosis and tricuspid  regurgitation. Management is intended for symptomatic relief, and valvular intervention is indicated in refractory heart failure. Balloon valvuloplasty is an option for patients not suitable for surgery. We report the case of a patient  with a carcinoid tumour, who developed postoperative refractory hypoxemia. Transthoracic echocardiogram revealed carcinoid pulmonary and tricuspid valve disease, with severe pulmonary stenosis. Balloon valvuloplasty was performed with major clinical improvement.

 

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[132]

TÍTULO / TITLE:  - Release of macrophage migration inhibitory factor by neuroendocrine differentiated LNCaP cells sustains the proliferation and survival of prostate cancer cells.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endocr Relat Cancer. 2012 Dec 3.

            ●● Enlace al texto completo (gratuito o de pago) 1530/ERC-12-0286

AUTORES / AUTHORS:  - Tawadros T; Alonso F; Jichlinski P; Clarke NW; Calandra T; Haefliger JA; Roger T

INSTITUCIÓN / INSTITUTION:  - T Tawadros, Urology, CHUV, Lausanne, Switzerland.

RESUMEN / SUMMARY:  - The acquisition of neuroendocrine (NE) characteristics by prostate cancer (PCa) cells is closely related to tumour progression and hormone-resistance. The mechanisms by which NE cells influence PCa growth and progression are not fully understood. Macrophage migration inhibitory factor (MIF) is a proinflammatory cytokine involved in oncogenic processes, and MIF serum levels correlate with aggressiveness of PCa. Here, we investigated the regulation and the functional consequences of MIF expression during NE transdifferentiation of PCa cells. NE differentiation of LNCaP cells, initiated either by increasing intracellular levels of cAMP or by culturing cells in an androgen depleted media, was associated with markedly increased MIF release. Yet, intracellular MIF protein and mRNA levels and MIF gene promoter activity decreased during NE differentiation of LNCaP cells, suggesting that NE differentiation favours MIF release despite decreasing MIF synthesis. Adenoviral-mediated forced MIF expression in NE differentiated LNCaP cells increased cell proliferation without  affecting the expression of NE markers. Addition of exogenous recombinant MIF to  LNCaP and PC-3 cells stimulated the AKT and ERK1/2 signalling pathways, the expression of genes involved in PCa, as well as proliferation and resistance to paclitaxel and thapsigargin-induced apoptosis. Altogether, these data provide evidences that increased MIF release during NE differentiation in PCa may facilitate cancer progression or recurrence, especially following androgen deprivation. Thus, MIF could represent an attractive target for PCa therapy.

 

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[133]

TÍTULO / TITLE:  - Frequency of multiple endocrine neoplasia type 1 in a group of patients with pituitary adenoma: genetic study and familial screening.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Pituitary. 2013 Jan 19.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s11102-013-0462-8

AUTORES / AUTHORS:  - Nunes VS; Souza GL; Perone D; Conde SJ; Nogueira CR

INSTITUCIÓN / INSTITUTION:  - Laboratory of Molecular Biology, Department of Internal Medicine, Botucatu Medical School, UNESP, Univ Estadual Paulista, Botucatu, Brazil, vsnunes@fmb.unesp.br.

RESUMEN / SUMMARY:  - The purpose of this study it was to evaluate the frequency of Multiple Endocrine  Neoplasia type 1 (MEN1) in patients with pituitary adenoma and to perform genetic analysis and familial screening of those individuals afflicted with MEN1. 144 patients with pituitary adenoma at Botucatu Medical School, UNESP-Univ Estadual Paulista, were assessed retrospectively for MEN1 during the years of 2005-2011. The patients were evaluated for the presence of primary hyperparathyroidism (PHP) and enteropancreatic tumors. Genetic analysis was performed for the individuals with clinically diagnosed MEN1. Thirteen patients met the diagnostic criteria for MEN1, but three individuals belong to the same family and they were considered as a single MEN1 event, revealing 7.7 % frequency of MEN1 in this patient group. Genetic analysis showed MEN1 mutations in four index cases: IVS4+1 G>A, IVS3-6 C>T, c.1547insC and a new D180A mutation. One patient did not agree to participate in the genetic study and another one was referred for follow up in other hospital. Only polymorphisms were found in the other individuals, one of which was novel. We identified a high frequency of MEN1 in pituitary adenoma patients. Since PHP is one of the most common MEN1 tumor and patients are mostly  asymptomatic, we suggest that all pituitary adenoma patients have their calcium profile analyzed.

 

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[134]

TÍTULO / TITLE:  - Insulinoma in a patient with type 2 diabetes mellitus proved at autopsy.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endocr Pract. 2012 Nov 1;18(6):1038.

AUTORES / AUTHORS:  - Kunieda T; Yamakita N; Yasuda K

INSTITUCIÓN / INSTITUTION:  - Department of General Internal Medicine, Matsunami General Hospital, Dendai 185-1 Kasamatsu-cho, Hashima-gun Gifu 501-6062, Japan.

 

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[135]

TÍTULO / TITLE:  - Esophageal small-cell cancer: study of a rare disease.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Dis Esophagus. 2013 Jan 14. doi: 10.1111/dote.12022.

            ●● Enlace al texto completo (gratuito o de pago) 1111/dote.12022

AUTORES / AUTHORS:  - Raja S; Rice TW; Rajeswaran J; Zhong J; Blackstone EH

INSTITUCIÓN / INSTITUTION:  - Department of Thoracic and Cardiovascular Surgery, Heart and Vascular Institute,  Cleveland Clinic, Cleveland, Ohio, USA.

RESUMEN / SUMMARY:  - Optimal treatment of esophageal small-cell cancer, a rare disease, lacks consensus. Based on its lung small-cell cancer analog, we hypothesized that chemotherapy with adjuvant radiotherapy would be optimal. This hypothesis was tested by studying the collective published literature. A meta-analysis of individual patients from 148 articles (1952-2010) explored treatment and outcome  of 577 patients with esophageal small-cell cancer. Hazard function frailty modeling identified optimum therapy after accounting for article-level and patient-level heterogeneity. Fifty-nine percent of publications reported one patient and 25% five or more. Sixty-six percent of patients were men, mean age was 63 +/- 11 years, and 64% had localized disease. One, 3-, and 5-year survival  was 37%, 14%, and 11%, respectively. Survival variation among articles was substantial (P = 0.004), with survival improving across time (P < 0.0004). Chemotherapy was associated with better survival (hazard ratio [HR] = 0.53, 68% confidence interval [CI] = 0.44-0.65; P = 0.002) than surgery alone, radiotherapy alone, nonstandard therapy, or no therapy. Adding local therapy, either surgery (HR = 0.41, 68% CI = 0.34-0.51; P < 0.0001) or radiotherapy (HR = 0.33, 68% CI =  0.27-0.41; P < 0.0001), to chemotherapy further improved survival. Adding both did not provide further benefit. The strategy of borrowing from consensus treatment of lung small-cell cancer and analyzing the scarce available esophageal small-cell cancer literature may be beneficial in the study of rare diseases. It  confirmed that chemotherapy should be the mainstay of therapy, with additional benefit from adjuvant therapy with either surgery or radiotherapy; both are not needed.

 

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[136]

TÍTULO / TITLE:  - Calcifying pseudoneoplasm of the neuraxis with single nerve rootlet involvement.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Can J Neurol Sci. 2012 Nov;39(6):840-2.

AUTORES / AUTHORS:  - Jentoft ME; Scheithauer BW; Bertoni F; Abood C; Chang HT

INSTITUCIÓN / INSTITUTION:  - Department of Laboratory Medicine and Pathology, 200 First Street, SW, Rochester, MN, 55905, USA. jentoft.mark@mayo.edu

 

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[137]

TÍTULO / TITLE:  - Bilateral pheochromocytomas in a child who had hemihypertrophy and alteration in  the VHL gene.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Pediatr Endocrinol Metab. 2013 Jan 17:1-4. doi: 10.1515/jpem-2012-0255.

            ●● Enlace al texto completo (gratuito o de pago) 1515/jpem-2012-0255

AUTORES / AUTHORS:  - Amini Z; Babovic-Vuksanovic D; Lteif A

RESUMEN / SUMMARY:  - Abstract The association of hypertrophy with neoplasm is well-known. Pheochromocytoma is a rare neoplasm in children. Isolated hemihypertrophy and hemihypertrophy linked to other genetic disorders have rarely been associated with the development of pheochromocytoma, with only two cases reported to date. We report a novel case of a 4-year-old male with bilateral adrenal pheochromocytomas and lower extremity hemihypertrophy in the setting of von Hippel-Lindau syndrome.

 

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[138]

TÍTULO / TITLE:  - Pancreatic Neuroendocrine Tumors: Signal Pathways and Targeted Therapies.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Curr Mol Med. 2013 Jan 15.

AUTORES / AUTHORS:  - Peng L; Schwarz RE

INSTITUCIÓN / INSTITUTION:  - Department of Pathology, UT Southwestern Medical Center, 5909 Harry Hines Boulevard, Dallas, TX 75390-9234, USA. lan.peng@utsouthwestern.edu.

RESUMEN / SUMMARY:  - Pancreatic neuroendocrine tumors (PNETs) are rare but are well understood to cover a broad spectrum of clinical presentation, tumor biology and prognosis. More than 60% of PNETs are diagnosed at advanced disease stage and are ineligible for surgical resection. Prior to 2011, streptozocin was the only approved agent for unresectable advanced PNETs. In recent years, breakthroughs in signal pathway research have led to the identification of new therapeutic targets and agents directed at the molecular level. In 2011, two new targeted therapeutic agents, sunitinib and everolimus, were approved by the Food and Drug Administration (FDA). Sunitinib is an inhibitor of multiple tyrosine kinases, and everolimus is  an inhibitor of the mammalian target of rapamycin (mTOR) pathway. This review discusses the major signaling pathways that are frequently mutated or deregulated in PNETs, and the implications of molecular alterations for PNET therapy. Biologic therapy through targeting relevant pathways represents a promising approach in the therapy of advanced and unresectable PNETs.

 

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[139]

TÍTULO / TITLE:  - Glucagon-like peptide-1 receptor imaging with [Lys(40)(Ahx-HYNIC- (99m)Tc/EDDA)NH (2)]-exendin-4 for the detection of insulinoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Eur J Nucl Med Mol Imaging. 2012 Dec 7.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s00259-012-2299-1

AUTORES / AUTHORS:  - Sowa-Staszczak A; Pach D; Mikolajczak R; Macke H; Jabrocka-Hybel A; Stefanska A; Tomaszuk M; Janota B; Gilis-Januszewska A; Malecki M; Kaminski G; Kowalska A; Kulig J; Matyja A; Osuch C; Hubalewska-Dydejczyk A

INSTITUCIÓN / INSTITUTION:  - Department of Endocrinology, Jagiellonian University Medical College, Kopernika 17, 31-501, Cracow, Poland, sowiana@gmail.com.

RESUMEN / SUMMARY:  - PURPOSE: The objective of this article is to present a new method for the diagnosis of insulinoma with the use of [Lys(40)(Ahx-HYNIC-(99m)Tc/EDDA)NH(2)]-exendin-4. METHODS: Studies were performed in 11 patients with negative results of all available non-isotopic diagnostic methods (8 with symptoms of insulinoma, 2 with malignant insulinoma and 1 with nesidioblastosis). In all patients glucagon-like peptide-1 (GLP-1) receptor imaging (whole-body and single photon emission computed tomography/CT examinations) after the injection of 740 MBq of the tracer was performed. RESULTS: Both sensitivity and specificity of GLP-1 receptor imaging were assessed to be 100 % in patients with benign insulinoma. In all eight cases with suspicion of insulinoma a focal uptake in the pancreas was found. In six patients surgical  excision of the tumour was performed (type G1 tumours were confirmed histopathologically). In one patient surgical treatment is planned. One patient was disqualified from surgery. In one case with malignant insulinoma pathological accumulation of the tracer was found only in the region of local recurrence. The  GLP-1 study was negative in the other malignant insulinoma patient. In one case with suspicion of nesidioblastosis, a focal accumulation of the tracer was observed and histopathology revealed coexistence of insulinoma and nesidioblastosis. CONCLUSION: [Lys(40)(Ahx-HYNIC-(99m)Tc/EDDA)NH(2)]-exendin-4 seems to be a promising diagnostic tool in the localization of small insulinoma tumours, but requires verification in a larger series of patients.

 

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[140]

TÍTULO / TITLE:  - Orbital carcinoid metastasis: diverse presentations and value of indium-octreotide imaging.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Orbit. 2012 Dec;31(6):379-82. doi: 10.3109/01676830.2012.711890.

            ●● Enlace al texto completo (gratuito o de pago) 3109/01676830.2012.711890

AUTORES / AUTHORS:  - Mehta P; Malik S; Adesanya O; Snead D; Ahluwalia H

INSTITUCIÓN / INSTITUTION:  - Department of Ophthalmology, University Hospitals of Coventry & Warwickshire NHS  Trust, Coventry, UK. purnima.mehta2@uhcw.nhs.uk

RESUMEN / SUMMARY:  - PURPOSE: To report two cases of orbital carcinoid metastasis (OCM) with diverse presentations and the role of Indium-Octreotide scan in management of these patients. METHODS: Clinical, histological and radiological findings and management of the two patients are described. Results are presented. CONCLUSIONS: We wish to highlight the diversity of presentations in orbital carcinoid. One patient presented with headache, while the other presented with recurrent transient ptosis. To the best of our knowledge, there have been no previous reports of OCM presenting as transient ptosis. The value of Indium-Octreotide scan in detecting the true extent of systemic disease and reducing the need for biopsy in carcinoid tumour is discussed.

 

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[141]

TÍTULO / TITLE:  - Gastrointestinal hormones stimulate growth of Foregut Neuroendocrine Tumors by transactivating the EGF receptor.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Biochim Biophys Acta. 2013 Mar;1833(3):573-82. doi: 10.1016/j.bbamcr.2012.11.021. Epub 2012 Dec 4.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.bbamcr.2012.11.021

AUTORES / AUTHORS:  - Di Florio A; Sancho V; Moreno P; Delle Fave G; Jensen RT

INSTITUCIÓN / INSTITUTION:  - Digestive Diseases Branch, NIDDK, National Institutes of Health, Bethesda, MD 20892-1804, USA.

RESUMEN / SUMMARY:  - Foregut neuroendocrine tumors [NETs] usually pursuit a benign course, but some show aggressive behavior. The treatment of patients with advanced NETs is marginally effective and new approaches are needed. In other tumors, transactivation of the EGF receptor (EGFR) by growth factors, gastrointestinal (GI) hormones and lipids can stimulate growth, which has led to new treatments. Recent studies show a direct correlation between NET malignancy and EGFR expression, EGFR inhibition decreases basal NET growth and an autocrine growth effect exerted by GI hormones, for some NETs. To determine if GI hormones can stimulate NET growth by inducing transactivation of EGFR, we examined the ability of EGF, TGFalpha and various GI hormones to stimulate growth of the human foregut carcinoid,BON, the somatostatinoma QGP-1 and the rat islet tumor,Rin-14B-cell lines. The EGFR tyrosine-kinase inhibitor, AG1478 strongly inhibited EGF and the  GI hormones stimulated cell growth, both in BON and QGP-1 cells. In all the three neuroendocrine cell lines studied, we found EGF, TGFalpha and the other growth-stimulating GI hormones increased Tyr(1068) EGFR phosphorylation. In BON cells, both the GI hormones neurotensin and a bombesin analogue caused a time- and dose-dependent increase in EGFR phosphorylation, which was strongly inhibited by AG1478. Moreover, we found this stimulated phosphorylation was dependent on Src kinases, PKCs, matrix metalloproteinase activation and the generation of reactive oxygen species. These results raise the possibility that disruption of this signaling cascade by either EGFR inhibition alone or combined with receptor  antagonists may be a novel therapeutic approach for treatment of foregut NETs/PETs.

 

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[142]

TÍTULO / TITLE:  - Bladder paraganglioma. A case report.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Med Liban. 2012 Jul-Sep;60(3):182-4.

AUTORES / AUTHORS:  - El Khoury F; Jour I; Malaeb B; Assaf G

INSTITUCIÓN / INSTITUTION:  - Urology Department, Saint George Hospital University Medical Center, University of Balamand, Beirut, Lebanon.

RESUMEN / SUMMARY:  - This is a report of a 32-year-old man who presented with dyspnea upon micturition. He was found to have hematuria. Pelvic ultrasound and CT scan of the abdomen and pelvis revealed a left bladder wall polyp confirmed by cystoscopy to  be a submucosal pulsating mass. Plasma free metanephrines were elevated in favor  of a bladder paraganlioma while urinary metanephrines, catecholamines and MIBG scan were normal. Patient was managed by partial cystectomy with disappearance of symptoms thereafter. Pathology showed a completely excised paraganglioma. The assessment, diagnosis and treatment are illustrated.

 

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[143]

TÍTULO / TITLE:  - Incipient Merkel Cell Carcinoma: A Report of 2 Cases.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Actas Dermosifiliogr. 2012 Dec 29. pii: S1578-2190(12)00360-5. doi: 10.1016/j.adengl.2012.11.007.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.adengl.2012.11.007

AUTORES / AUTHORS:  - Requena C; Traves V; Llombart B; Guillen C

INSTITUCIÓN / INSTITUTION:  - Servicio de Dermatologia, Instituto Valenciano de Oncologia, Valencia, España. Electronic address: celiareq@hotmail.com.

RESUMEN / SUMMARY:  - Merkel cell carcinoma is a malignant skin tumor with a poor prognosis that primarily affects photoexposed areas of elderly patients. Tumor size is a very strong prognostic factor, with much better outcomes associated with small lesions, measuring less than 1cm. However, such lesions are rarely seen in the clinic in view of the rapid growth of this tumor. We report 2 cases of incipient  Merkel cell carcinoma. Both cases of incipient Merkel cell carcinoma measured approximately 5mm in diameter. One tumor was confined to the epidermis and papillary dermis on the nose of a 79-year-old man and the other was located in the deep dermis, almost in the hypodermis, on the buttock of an 82-year-old woman. In both cases, the lesions had appeared weeks earlier. The first tumor seemed to originate in the dermoepidermal junction whereas the second originated  almost in the hypodermis. Although the lesions were at a similar disease stage and had a similar size, their different locations within the dermis highlight once again the controversy about which cells give rise to Merkel cell carcinoma.  The precursor cells could feasibly be Merkel cells in the first case but not in the second.

 

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[144]

TÍTULO / TITLE:  - Multiple carcinoid tumors of the small intestine preoperatively diagnosed by double-balloon endoscopy.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Med Sci Monit. 2012 Dec 1;18(12):CS109-112.

AUTORES / AUTHORS:  - Lee SY; Tomoyoshi S; Haga K; Sasaki H; Ogata C; Nomura O; Fukuo Y; Abe W; Osada T; Nagahara A; Ogihara T; Kamiyama H; Sakamoto K; Watanabe S

INSTITUCIÓN / INSTITUTION:  - Department of Gastroenterology, Juntendo University School of Medicine, Tokyo, Japan.

RESUMEN / SUMMARY:  - Background: Multiple carcinoid tumors of the small intestine are rare and are very difficult to detect preoperatively. Case Report: A 75-year-old woman in whom the bleeding focus could not be found by upper and lower endoscopy and abdominal  CT was admitted for evaluation of anemia. We examined the patient with total double-balloon endoscopy (DBE) and located multiple submucosal tumors. The multiple carcinoid tumors were resected successfully under laparoscopy. Conclusions: We report a case of a successful laparoscopic operation for multiple carcinoid tumors in the small intestine without intraoperative endoscopy. Total digestive tract observation using DBE is very useful for laparoscopic operation for multiple tumors in the small intestine.

 

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[145]

TÍTULO / TITLE:  - Molecular Epidemiology of Multiple Endocrine Neoplasia 2: Implications for RET Screening in the New Millenium.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Eur J Endocrinol. 2012 Dec 4.

            ●● Enlace al texto completo (gratuito o de pago) 1530/EJE-12-0919

AUTORES / AUTHORS:  - Machens A; Lorenz K; Sekulla C; Hoeppner W; Frank-Raue K; Raue F; Dralle H

INSTITUCIÓN / INSTITUTION:  - A Machens, Department of General, Visceral and Vascular Surgery, Martin Luther University Halle-Wittenberg, Halle (Saale), D-06097, Germany.

RESUMEN / SUMMARY:  - OBJECTIVETWENTY YEARS AGO, THE GROUND-BREAKING DISCOVERY THAT REARRANGED DURING TRANSFECTION (RET) MUTATIONS UNDERLIE MULTIPLE ENDOCRINE NEOPLASIA 2 (MEN 2) AND  FAMILIAL MEDULLARY THYROID CANCER (FMTC) USHERED IN THE ERA OF PERSONALIZED MEDICINE. MEN2-ASSOCIATED SIGNS, TAKING TIME TO MANIFEST, CAN BE SUBTLE. THIS STUDY SOUGHT TO CLARIFY TO WHAT EXTENT CONVENTIONAL ESTIMATES OF 1:200,000-500,000 UNDERESTIMATE THE INCIDENCE OF RET MUTATIONS IN THE POPULATION.DESIGNINCLUDED IN THIS RETROSPECTIVE INVESTIGATION WERE 333 RET CARRIERS BORN BETWEEN 1951 AND 2000 AND OPERATED ON AT THE LARGEST GERMAN SURGICAL REFERRAL CENTER (286 CARRIERS) OR ELSEWHERE (47 CARRIERS).METHODSTO ESTIMATE THE INCIDENCE OF RET MUTATIONS, THE NUMBER OF RET CARRIERS BORN IN GERMANY IN FIVE DECADES (1951-1960, 1961-1970, 1971-1980, 1981-1990, AND 1991-2000) WAS DIVIDED BY THE CORRESPONDING NUMBER OF GERMAN LIVE BIRTHS.RESULTSOWING TO IMPROVED DIAGNOSIS AND CAPTURE OF FMTC AND MEN2 PATIENTS,  MINIMUM INCIDENCE ESTIMATES INCREASED OVER TIME: overall from 5.0 (1951-1960) to  9.9 (1991-2000) per million live births and year (P=0.008), and by American Thyroid Association/ATA class from 1.7 to 3.7 for ATA class C (P=0.008); from 1.8 to 2.7 for ATA class A (P=0.017); from 1.5 to 2.2 for ATA class B (P=0.20); and from 0 to 1.4 for ATA class D mutations per million live births and year (P=0.008). Based on 1991-2000 incidence estimates, prevalence in Germany approximates 1:80,000 inhabitants.ConclusionsThe molecular minimum incidence estimate of approximately 1:100,000 was 2-5-fold greater than conventional estimates of 1:200,000-500,000.

 

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[146]

TÍTULO / TITLE:  - (68)Ga-DOTA-TOC uptake in neuroendocrine tumour and healthy tissue: differentiation of physiological uptake and pathological processes in PET/CT.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Eur J Nucl Med Mol Imaging. 2013 Jan 5.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s00259-012-2309-3

AUTORES / AUTHORS:  - Kroiss A; Putzer D; Decristoforo C; Uprimny C; Warwitz B; Nilica B; Gabriel M; Kendler D; Waitz D; Widmann G; Virgolini IJ

INSTITUCIÓN / INSTITUTION:  - Department of Nuclear Medicine, Innsbruck Medical University, Anichstrasse 35, 6020, Innsbruck, Austria, alexander.kroiss@i-med.ac.at.

RESUMEN / SUMMARY:  - PURPOSE: We wanted to establish the range of (68)Ga-DOTA-TOC uptake in liver and  bone metastases of patients with neuroendocrine tumours (NET) and to establish the range of its uptake in pancreatic NET. This would allow differentiation between physiological uptake and tumour-related somatostatin receptor expression  in the pancreas (including the uncinate process), liver and bone. Finally, we wanted to test for differences in patients with NET, either treated or not treated with peptide receptor radionuclide therapy (PRRT). METHODS: In 249 patients, 390 (68)Ga-DOTA-TOC PET/CT studies were performed. The clinical indications for PET/CT were gastroenteropancreatic NET (194 studies), nongastroenteropancreatic NET (origin in the lung and rectum; 46 studies), NET of unknown primary (111 studies), phaeochromocytoma/glomus tumours (18 studies), and radioiodine-negative metastatic thyroid carcinoma (21 studies). RESULTS: SUV(max) (mean +/- standard deviation) values of (68)Ga-DOTA-TOC were 29.8 +/- 16.5 in 162 liver metastases, 19.8 +/- 18.8 in 89 bone metastases and 34.6 +/- 17.1 in 43 pancreatic NET (33.6 +/- 14.3 in 30 tumours of the uncinate process and 36.3 +/-  21.5 in 13 tumours of the pancreatic tail). A significant difference in SUV(max)  (p < 0.02) was found in liver metastases of NET patients treated with PRRT. There were significant differences in SUV(max) between nonmalignant and malignant tissue for both bone and liver metastases and for pancreatic NET including the uncinate process (p < 0.0001). At a cut-off value of 17.1 the specificity and sensitivity of SUV(max) for differentiating tumours in the uncinate process were  93.6 % and 90.0 %, respectively (p < 0.0001). CONCLUSION: (68)Ga-DOTA-TOC is an excellent tracer for the imaging of tumours expressing somatostatin receptors on  the tumour cell surface, facilitating the detection of even small tumour lesions. The noninvasive PET/CT approach by measurement of regional SUV(max) can offer important clinical information to distinguish between physiological and pathological somatostatin receptor expression, especially in the uncinate process. PRRT does not significantly influence SUV(max), except in liver metastases of patients with NET.

 

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[147]

TÍTULO / TITLE:  - Gastric undifferentiated carcinoma with diffuse c-kit overexpression and focal neuroendocrine differentiation.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Pathol Res Pract. 2013 Jan 21. pii: S0344-0338(12)00332-9. doi: 10.1016/j.prp.2012.12.004.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.prp.2012.12.004

AUTORES / AUTHORS:  - Mori D; Akashi M; Baba K; Morito K; Shibaki M; Hashimoto M; Nakamura A; Mawatari S; Sato S

INSTITUCIÓN / INSTITUTION:  - Division of Pathology, Saga Prefectural Hospital, Koseikan, Saga, Japan. Electronic address: mori-d@koseikan.jp.

RESUMEN / SUMMARY:  - A case of gastric undifferentiated carcinoma with diffuse c-kit overexpression and focal neuroendocrine differentiation is described. A 76-year-old man presented appetite loss for 1 month. Gastric endoscopy showed an exophytic huge nodular mass with central ulceration at the gastric prepylorus. Distal gastrectomy was performed with lymph node dissection. Histology indicated anaplastic medium- to large-sized round tumor cells in discohesive sheets. Adenocarcinomatous areas forming tubular glands or with intracytoplasmic mucin on PAS and Alcian-blue staining were not found in any sections. Immunohistochemistry showed that the tumor cells were diffusely positive for cytokeratin, vimentin, c-kit and focally positive for chromogranin A and synaptophysin. We hypothesized  that c-kit overexpression of this tumor was attributed to neuroendocrine differentiation.

 

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[148]

TÍTULO / TITLE:  - Epidemiology of Malignant Digestive NeuroEndocrine Tumours (MD-NET).

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Eur J Endocrinol. 2013 Jan 24.

            ●● Enlace al texto completo (gratuito o de pago) 1530/EJE-12-0418

AUTORES / AUTHORS:  - Lepage C; Bouvier AM; Faivre J

INSTITUCIÓN / INSTITUTION:  - C Lepage, Digestive Cancer Registry, INSERM U866, University of Burgundy, Dijon,  France.

RESUMEN / SUMMARY:  - Little is known about patients with malignant digestive neuroendocrine tumours (MD-NETs). Although their incidence is increasing, MD-NETs remain a rare cancer,  representing 1% of digestive cancers. Most MD-NETs are well-differentiated. MD-NET poorly differentiated carcinomas account for 20% of cases on average. Anatomical localisation of MD-NETs varied according to geographic region. Stage at diagnosis and prognosis for patients with MD-NETs in the general population are considerably worse than often reported from small hospital case series. Prognosis varies with tumour differentiation, anatomic site, and histological subtype. There are significant differences in survival from MD-NETs among European countries, independent of other prognostic factors. Early diagnosis is difficult; new therapeutic options appear to represent the best approach to improving prognosis.

 

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[149]

TÍTULO / TITLE:  - Paraganglioma of the maxillary sinus.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Auris Nasus Larynx. 2012 Dec 18. pii: S0385-8146(12)00228-3. doi: 10.1016/j.anl.2012.10.009.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.anl.2012.10.009

AUTORES / AUTHORS:  - Kisser U; Braun T; Mayr D; Leunig A

INSTITUCIÓN / INSTITUTION:  - Department of Otorhinolaryngology, Head and Neck Surgery, University of Munich, Germany. Electronic address: Ulrich.Kisser@med.uni-muenchen.de.

RESUMEN / SUMMARY:  - Primary paragangliomas of the paranasal sinuses are very rare conditions with only few cases described in the literature. Paragangliomas are locally aggressive, often recur and can metastasize. Usually, open surgery is used to resect such tumors from the sinonasal tract. Here, a case of a large paraganglioma of the left maxillary sinus and nasal cavity, which was successfully removed using the Onyx(®) embolic agent two days prior to minimally invasive image guided endoscopic sinus surgery, is reported. This case  demonstrates that large vascular tumors of the sinonasal tract can be successfully managed by endoscopic endonasal sinus surgery. The patient has no evidence of recurrence after 12 months of follow-up.

 

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[150]

TÍTULO / TITLE:  - Biphasic large cell neuroendocrine carcinoma—pure mucinous carcinoma of the gallbladder (MANEC): a unique combination.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Pathologica. 2012 Aug;104(4):185-9.

AUTORES / AUTHORS:  - Russo S; Russo F; Maiello FM; Paolini B; Carrabba A; De Gregorio A

INSTITUCIÓN / INSTITUTION:  - Pathology Dpt., Maresca Hospital, Torre del Greco, Napoli, Italy. qubmur@tin.it

RESUMEN / SUMMARY:  - INTRODUCTION: We report a case of primary combined large cell neuroendocrine carcinoma (LCNEC)--pure mucinous carcinoma of the gallbladder (MANEC)--which represents the first description of this entity. METHODS: The patient is a 59-year-old Italian male who underwent cholecystectomy under a preoperative diagnosis of cholecystitis with gallstones and gallbladder tumour. During laparotomy, cholecystectomy, liver wedge resection and regional lymph node dissection were performed. The resected gallbladder showed a thickened wall, gallstones and a 4 cm gelatinous, cauliflower-like soft tissue mass. RESULTS: Following surgery, the gallbladder tumour was diagnosed as a mixed endocrine-exocrine carcinoma. There was evidence of lymph node metastasis or direct liver invasion. The mucin-producing carcinoma was composed of poorly differentiated glandular cells with mucin lakes. The LCNEC was characterized by large cells with prominent nucleoli, coarse chromatin and a high mitotic rate. The cells showed an “organoid” growth pattern with rosette formation and frequent areas of necrosis. Chromogranin A, synaptophysin and CD56 were diffusely and strongly expressed. DISCUSSION: This case may provide helpful insights regarding  the histogenesis of this unusual combination of tumors: the concept of a collision tumor between two neoplasms that have arisen in adjacent areas may be the best explanation for its pathogenesis.

 

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[151]

TÍTULO / TITLE:  - Evaluation of intratumoural heterogeneity on (18)F-FDG PET/CT for characterization of peripheral nerve sheath tumours in neurofibromatosis type 1.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Eur J Nucl Med Mol Imaging. 2012 Dec 12.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s00259-012-2314-6

AUTORES / AUTHORS:  - Salamon J; Derlin T; Bannas P; Busch JD; Herrmann J; Bockhorn M; Hagel C; Friedrich RE; Adam G; Mautner VF

INSTITUCIÓN / INSTITUTION:  - Department of Diagnostic and Interventional Radiology, University Medical Center  Hamburg-Eppendorf, Martinistr. 52, 20246, Hamburg, Germany, j.salamon@uke.de.

RESUMEN / SUMMARY:  - PURPOSE: The aim of the study was to evaluate the potential usefulness of intratumoural tracer uptake heterogeneity on (18)F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT as compared to a cut-off maximum standardized uptake value (SUV(max)) for characterization of peripheral nerve sheath tumours (PNSTs) in neurofibromatosis type 1 (NF1). METHODS: Fifty patients suffering from NF1 were examined by (18)F-FDG PET/CT. Intralesional tracer uptake was analysed qualitatively and semi-quantitatively by measuring the mean and maximum SUV. Uptake heterogeneity was graded qualitatively using a three-point scale and semi-quantitatively by calculating an SUV-based heterogeneity index (HI(SUV)). Cohen’s kappa was used to determine inter- and intra-rater agreement.  Histopathological evaluation and clinical as well as radiological follow-up examinations served as the reference standards. RESULTS: A highly significant correlation between the degree of intratumoural uptake heterogeneity on (18)F-FDG PET and malignant transformation of PNSTs was observed (p < 0.0001). Semi-quantitative HI(SUV) was significantly higher in malignant PNSTs (MPNSTs) than in benign tumours (p = 0.0002). Both intralesional heterogeneity and SUV(max) could be used to identify malignant tumours with a sensitivity of 100 %. Cohen’s kappa was 0.86 for inter-rater agreement and 0.88 for intra-rater agreement on heterogeneity. CONCLUSION: MPNSTs in patients with NF1 demonstrate considerable intratumoural uptake heterogeneity on (18)F-FDG PET/CT. Assessment of tumour heterogeneity is highly reproducible. Both tumour heterogeneity and a cut-off SUV(max) may be used to sensitively identify malignant PNSTs, but the specificity is higher for the latter. A combination of both methods leads to a non-significant improvement in diagnostic performance.

 

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[152]

TÍTULO / TITLE:  - Angiofibromas in multiple endocrine neoplasia type 1.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Dermatol Online J. 2012 Dec 15;18(12):20.

AUTORES / AUTHORS:  - Vashi N; Hunt R; Fischer M; Meehan S; Pomeranz MK

INSTITUCIÓN / INSTITUTION:  - The Ronald O. Perelman Department of Dermatology, New York University School of Medicine.

RESUMEN / SUMMARY:  - Multiple endocrine neoplasia type 1 (MEN1) is a familial tumor syndrome with autosomal dominant inheritance. Cutaneous tumors in MEN1, which include multiple  angiofibromas, collagenomas, and lipomas can easily be overlooked because of their subtle appearance. As markers of this tumor syndrome, recognition of the mucocutaneous manifestations of MEN1 is important in order to facilitate early interdisciplinary care and diagnosis of associated internal disease in both patients and family members.

 

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[153]

TÍTULO / TITLE:  - Mitochondrial complex II and genomic imprinting in inheritance of paraganglioma tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Biochim Biophys Acta. 2013 Jan 2. pii: S0005-2728(12)01105-X. doi: 10.1016/j.bbabio.2012.12.005.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.bbabio.2012.12.005

AUTORES / AUTHORS:  - Baysal BE

INSTITUCIÓN / INSTITUTION:  - Department of Pathology, Roswell Park Cancer Institute, Buffalo, NY, 14263, USA.  Electronic address: bora.baysal@roswellpark.org.

RESUMEN / SUMMARY:  - Germ line heterozygous mutations in the structural subunit genes of mitochondrial complex II (succinate dehydrogenase; SDH) and the regulatory gene SDHAF2 predispose to paraganglioma tumors which show constitutive activation of hypoxia  inducible pathways. Mutations in SDHD and SDHAF2 cause highly penetrant multifocal tumor development after a paternal transmission, whereas maternal transmission rarely, if ever, leads to tumor development. This transmission pattern is consistent with genomic imprinting. Recent molecular evidence supports a model for tissue-specific imprinted regulation of the SDHD gene by a long range epigenetic mechanism. In addition, there is evidence of SDHB mRNA editing in peripheral blood mononuclear cells and long-term balancing selection operating on the SDHA gene. Regulation of SDH subunit expression by diverse epigenetic mechanisms implicates a crucial dosage-dependent role for SDH in oxygen homeostasis. This article is part of a Special Issue entitled: Respiratory complex II: Role in cellular physiology and disease.

 

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[154]

TÍTULO / TITLE:  - Somatostatin-based radiotherapy with [90Y-DOTA]-TOC in neuroendocrine tumors: long-term outcome of a phase I dose escalation study.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Transl Med. 2013 Jan 15;11(1):17.

            ●● Enlace al texto completo (gratuito o de pago) 1186/1479-5876-11-17

AUTORES / AUTHORS:  - Marincek N; Jorg AC; Brunner P; Schindler C; Koller MT; Rochlitz C; Muller-Brand J; Maecke HR; Briel M; Walter MA

RESUMEN / SUMMARY:  - ABSTRACT: BACKGROUND: We describe the long-term outcome after clinical introduction and dose escalation of somatostatin receptor targeted therapy with [90Y-DOTA]-TOC in patients with metastasized neuroendocrine tumors. METHODS: In a clinical phase I dose escalation study we treated patients with increasing [90Y-DOTA]-TOC activities. Multivariable Cox regression and competing risk regression were used to compare efficacy and toxicities of the different dosage protocols. RESULTS: Overall, 359 patients were recruited; 60 patients were enrolled for low dose (median: 2.4 GBq/cycle, range 0.9-7.8 GBq/cycle), 77 patients were enrolled for intermediate dose (median: 3.3 GBq/cycle, range: 2.0-7.4 GBq/cycle) and 222 patients were enrolled for high dose (median: 6.7 GBq/cycle, range: 3.7-8.1 GBq/cycle) [90Y-DOTA]-TOC treatment. The incidences of  hematotoxicities grade 1--4 were 65.0%, 64.9% and 74.8%; the incidences of grade  4/5 kidney toxicities were 8.4%, 6.5% and 14.0%, and the median survival was 39 (range: 1--158) months, 34 (range: 1--118) months and 29 (range: 1--113) months.  The high dose protocol was associated with an increased risk of kidney toxicity (Hazard Ratio: 3.12 (1.13-8.59) vs. intermediate dose, p = 0.03) and a shorter overall survival (Hazard Ratio: 2.50 (1.08-5.79) vs. low dose, p = 0.03). CONCLUSIONS: Increasing [90Y-DOTA]-TOC activities may be associated with increasing hematological toxicities. The dose related hematotoxicity profile of [90Y-DOTA]-TOC could facilitate tailoring [90Y-DOTA]-TOC in patients with preexisting hematotoxicities. The results of the long-term outcome suggest that fractionated [90Y-DOTA]-TOC treatment might allow to reduce renal toxicity and to improve overall survival. (ClinicalTrials.gov number NCT00978211).

 

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[155]

TÍTULO / TITLE:  - Concurrent AURKA and MYCN Gene Amplifications Are Harbingers of Lethal Treatment-Related Neuroendocrine Prostate Cancer.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Neoplasia. 2013 Jan;15(1):1-10.

AUTORES / AUTHORS:  - Mosquera JM; Beltran H; Park K; Macdonald TY; Robinson BD; Tagawa ST; Perner S; Bismar TA; Erbersdobler A; Dhir R; Nelson JB; Nanus DM; Rubin MA

INSTITUCIÓN / INSTITUTION:  - Weill Cornell Cancer Center, Weill Medical College of Cornell University, New York, NY ; Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University, New York, NY.

RESUMEN / SUMMARY:  - Neuroendocrine prostate cancer (NEPC), also referred to as anaplastic prostate cancer, is a lethal tumor that most commonly arises in late stages of prostate adenocarcinoma (PCA) with predilection to metastasize to visceral organs. In the  current study, we explore for evidence that Aurora kinase A (AURKA) and N-myc (MYCN) gene abnormalities are harbingers of treatment-related NEPC (t-NEPC). We studied primary prostate tissue from 15 hormone naive PCAs, 51 castration-resistant prostate cancers, and 15 metastatic tumors from 72 patients  at different stages of disease progression to t-NEPC, some with multiple specimens. Histologic evaluation, immunohistochemistry, and fluorescence in situ  hybridization were performed and correlated with clinical variables. AURKA amplification was identified in overall 65% of PCAs (hormone naive and treated) from patients that developed t-NEPC and in 86% of metastases. Concurrent amplification of MYCN was present in 70% of primary PCAs, 69% of treated PCAs, and 83% of metastases. In contrast, in an unselected PCA cohort, AURKA and MYCN amplifications were identified in only 5% of 169 cases. When metastatic t-NEPC was compared to primary PCA from the same patients, there was 100% concordance of ERG rearrangement, 100% concordance of AURKA amplification, and 60% concordance of MYCN amplification. In tumors with mixed features, there was also 100% concordance of ERG rearrangement and 94% concordance of AURKA and MYCN co-amplification between areas of NEPC and adenocarcinoma. AURKA and MYCN amplifications may be prognostic and predictive biomarkers, as they are harbingers of tumors at risk of progressing to t-NEPC after hormonal therapy.

 

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[156]

TÍTULO / TITLE:  - Multiple gastrointestinal stromal tumors and pheochromocytoma in a patient with von Recklinghausen’s disease.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Int J Surg Case Rep. 2013;4(2):216-8. doi: 10.1016/j.ijscr.2012.10.024. Epub 2012 Nov 23.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.ijscr.2012.10.024

AUTORES / AUTHORS:  - Ozcinar B; Aksakal N; Agcaoglu O; Tukenmez M; Ozemir IA; Barbaros U; Colak N; Erbil Y

INSTITUCIÓN / INSTITUTION:  - Istanbul University, Istanbul Medical Faculty, General Surgery Department, Capa,  Istanbul, Turkey. Electronic address: drbeyza@hotmail.com.

RESUMEN / SUMMARY:  - INTRODUCTION: Neurofibromatosis type 1 is a genetic disease characterized by neoplastic and non neoplastic disorders involving tissues of neuroectodermal and  mesenchymal origin. Herein, we present a case with von Recklinghausen’s disease,  right adrenal heochromocytoma and multiple gastrointestinal stromal tumors. PRESENTATION OF CASE: A forty-eight year old male patient was admitted to our Emergency Department with melena. His physical examination revealed multiple neurofibromas all over the skin, kyphosis, multiple cafe au lait spots and Lisch  nodules on the eye and, melena on digital rectal examination. Abdominal computerized tomography scan showed a mass on right adrenal gland and multiple soft tissue mass lesions between distal part of pancreas and small bowel. Adrenal mass was determined as a pheochromocytoma and small bowel lesions were verified as stromal tumors. DISCUSSION: In patients with NF1, pheochromocytomas and GISTs  are well known neoplasms seen with increased incidence than the general population. CONCLUSION: In patients with NF1, any symptoms with other systems should be managed carefully for underlying malignity.

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[157]

TÍTULO / TITLE:  - Oral agents for treatment of patients with advanced pancreatic neuroendocrine tumors: could pharmaeconomic, cost-effectiveness data play a significant role?

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - JOP. 2013 Jan 10;14(1):102-4. doi: 10.6092/1590-8577/1354.

AUTORES / AUTHORS:  - Barna ME; Uomo I; Pastorello M

INSTITUCIÓN / INSTITUTION:  - Department of Pharmacy, Local Health Unit. Palermo, Italy. ilariauomo@libero.it.

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[158]

TÍTULO / TITLE:  - Pheochromocytoma revealed by acute heart failure. When should we operate? Presented at the ESES Congress, Gothenburg May 25-26, 2012.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Langenbecks Arch Surg. 2012 Dec 19.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s00423-012-1040-7

AUTORES / AUTHORS:  - Muller G; Saint F; Hamy A; Lifante JC; Carnaille B; Sebag F; Menegaux F; Vandwalle J; Drui D; Caillard C; Rodien P; Roy M; Peix JL; Pattou F; Brunaud L; Henry JF; Mirallie E

INSTITUCIÓN / INSTITUTION:  - Department of Digestive and Endocrine Surgery, CCDE, IMAD, CHU Nantes, Nantes, France.

RESUMEN / SUMMARY:  - PURPOSE: The aim of this study was to assess the safety and efficacy of adrenalectomy on patients with pheochromocytoma diagnosed at the time of an acute heart failure (AHF). METHODS: We reported cases of patients who presented an AHF  secondary to a pheochromocytoma during a period of 10 years. The diagnosis of AHF was defined by a left ventricular ejection fraction of less than 30 % or the use  of circulatory assistance. They had adrenalectomy as emergency surgery or later.  Morbidity and mortality of surgery were studied. RESULTS: Thirteen patients required an adrenalectomy for AHF secondary to pheochromocytoma. Four patients (31 %) had an adrenalectomy in emergency. Nine patients (69 %) had a delayed surgery with a median delay of 25 days (7-180). Eight patients had circulatory assistance (61 %). Five of them had a circulatory assistance and a delayed surgery (38 %), two of them had a circulatory assistance followed by emergency surgery (at 1.5 and 3 days) and one had emergency surgery immediately followed by circulatory assistance. Emergency surgery was performed by laparotomy in all cases and delayed surgery by laparoscopy for seven patients (54 %). Perioperative complications consisted in: one circulatory arrest, two bleedings requiring transfusion, one intestinal ischaemia, one haemoperitoneum with re-operation (day 8). One patient died on day 5. Post-operative course of patients with delayed surgery was uneventful. CONCLUSIONS: AHF revealing a pheochromocytoma is a rare and serious event. Patients with emergency surgery have more complications than those with delayed surgery.

 

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[159]

TÍTULO / TITLE:  - Questions and answers: what can be said by diagnostic imaging in neuroendocrine tumors?

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Minerva Endocrinol. 2012 Dec;37(4):367-77.

AUTORES / AUTHORS:  - Cuccurullo V; Faggiano A; Scialpi M; Cascini GL; Piunno A; Catalano O; Colao A; Mansi L

INSTITUCIÓN / INSTITUTION:  - Department of Nuclear Medicine, Seconda Universita degli Studi, Naples, Italy - vincenzo.cuccurullo@unina2.it.

RESUMEN / SUMMARY:  - The neuroendocrine tumors (NET) of the gastro-entero-pancreatic area (GEP) represent a heterogeneous group of malignancies from the histologic, clinico-laboratoristic (functioning and non-functioning variants), and therapeutic point of view. It is an issue becoming more frequent for the diagnostic imager, being radiologist as well as nuclear physician. Imaging (together with biopsy) plays a key role in the diagnostic assessment and staging  (including grading and prognostic definition), in evaluating response to treatment, and in follow-up of GEP-NET. Multislice computed tomography (MSCT), octreoscan and PET-CT are the most widely diffuse and accurate imaging modalities employed in this setting. Other methods, such as Magnetic Resonance and Endoscopic Ultrasound, may also play a significant role.

 

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[160]

TÍTULO / TITLE:  - Calcitonin and procalcitonin in patients with medullary thyroid cancer or bacterial infection.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Adv Clin Exp Med. 2012 Mar-Apr;21(2):169-78.

AUTORES / AUTHORS:  - Kaczka K; Mikosinski S; Fendler W; Celnik A; Pomorski L

INSTITUCIÓN / INSTITUTION:  - Department of General and Oncological Surgery, Medical University of Lodz, Poland. krzysztofkaczka@poczta.fm

RESUMEN / SUMMARY:  - OBJECTIVES: To evaluate procalcitonin (PCT) utility as a marker of medullary thyroid cancer (MTC). MATERIAL AND METHODS: Calcitonin (CT) and PCT levels were measured in MTC patients and patients with serious bacterial infections. 70 patients were enrolled in the study: 6 MTC active patients: 4 with disseminated,  unreoperable disease and 2 re-operated patients, in whom markers were checked before and after surgery; 23 MTC patients in remission after radical surgery; 11  non-toxic nodular goiter (NTNG) patients; 30 patients with severe, bacterial infection or sepsis. RESULTS: All MTC active patients had greatly elevated CT and PCT levels. In two re-operated patients, marker levels decreased but were still above the reference range. In 15 MTC patients in remission, the levels of either  marker were not increased. Both markers were slightly increased in 3 patients in  this group, while CT was elevated in 5 patients. In all but 1 patient in the NTNG group, both marker levels were not elevated. Among patients with bacterial infection, PCT and CT levels showed no increase in 8 patients, both markers were  elevated in 10 patients, and an increase of PCT levels was seen in 10 patients while of CT only in 2 patients. Correlations between CT and PCT values were very  strong in MTC patients (r = 0.95; p = 0.004 for active MTC, r = 0.60; p = 0.002 for MTC patients in remission) and in patients with NTNG (r = 0.77; p = 0.02). In patients with infection, both parameters were completely independent (r = 0.002;  p = 0.99). CONCLUSIONS: PCT measurement could be an alternative to CT measurement for evaluation of MTC status.

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[161]

TÍTULO / TITLE:  - Surgical management of medullary thyroid cancer.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Minerva Endocrinol. 2012 Dec;37(4):329-34.

AUTORES / AUTHORS:  - Mazeh H; Sippel RS

INSTITUCIÓN / INSTITUTION:  - Section of Endocrine Surgery, Department of Surgery, University of Wisconsin, Madison, WI, USA - sippel@surgery.wisc.edu.

RESUMEN / SUMMARY:  - Although thyroid cancer accounts for only 1.5% of all malignancies in the US it is the most rapidly increasing cancer in incidence and it is the most common endocrine malignancy that accounts for over 95% of the endocrine malignancies. Medullary thyroid cancer (MTC) originates from the parafollicular C cells and it  represents 6-8% of all thyroid cancer cases. As many as 25% of the MTCs are familial and carry a specific germline mutation as compared to only than 10% familial inheritance in non-medullary thyroid cancers. While well-differentiated  thyroid malignancies carry a very good prognosis, recurrence and survival rates of patients with MTC are significantly worse. The difference in cell origin and differentiation also results in different available adjunct therapy. The aim of this study is to review in detail the surgical management of patients with MTC.

 

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[162]

TÍTULO / TITLE:  - Is determination of matrix metalloproteinases and their tissue inhibitors serum concentrations useful in patients with gastroenteropancreatic and bronchopulmonary neuroendocrine neoplasms?

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endokrynol Pol. 2012;63(6):470-6.

AUTORES / AUTHORS:  - Blicharz-Dorniak J; Kos-Kudla B; Foltyn W; Kajdaniuk D; Marek B; Zemczak A; Strzelczyk J

RESUMEN / SUMMARY:  - Introduction: Gastroenteropancreatic (GEP) and bronchopulmonary (BP) neurendocrine neoplasms (NENs) are rare and slowly growing tumours. Matrix metalloproteinases (MMPs) degrade extracellular matrix and are responsible for invasion and metastasis. Tissue inhibitors of matrix metalloproteinases (TIMPs) affect the invasiveness of tumour cells and the formation of distant metastases.  The aim of this study was to evaluate selected MMPs (MMP2 and MMP9) and their tissue inhibitors (TIMP1 and TIMP2) depending on the pTNM classification, grading, and the occurrence of metastases. Material and methods: The study group  consisted of 86 patients with GEP NENs. The control group consisted of 31 healthy volunteers. Serum levels of TIMP1, TIMP2, MMP2 and MMP9 were determined by ELISA  (R&D Systems) in all the study subjects. The statistical calculations were performed using MedCalc. Results: We observed significant differences in MMP2 and TIMP1 levels between the study group with NENs and the control group. TIMP1 levels were significantly higher in patients with high-grade NEN (NEC, neuroendocrine carcinoma) compared to patients with low-grade tumour (NET G1, neuroendocrine tumours G1) (p < 0.017). We also observed a significant correlation between TIMP1 levels and the presence of metastases in the group of patients with GEP NENs, and also higher TIMP1 levels than those in the patients without metastases (p < 0.05). We also found a higher likelihood of metastases in patients with GEP NENs with TIMP1 levels exceeding 206.4 ng/mL. Conclusions: Patients with NENs secreted larger quantities of MMP2 and TIMP1. TIMP1 may be considered a marker of metastases in patients with GEP NENs. (Endokrynol Pol 2012; 63 (6): 470-476).

 

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[163]

TÍTULO / TITLE:  - Pancreatic neuroendocrine neoplasms.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Minerva Gastroenterol Dietol. 2012 Dec;58(4):401-26.

AUTORES / AUTHORS:  - Horsch D; Bert T; Schrader J; Hommann M; Kaem-Merer D; Petrovitch A; Zaknun J; Baum RP

INSTITUCIÓN / INSTITUTION:  - Center for Neuroendocrine Tumors Bad Berka ENETS Center of excellence, Bad Berka, Germany - dieter.hoersch@zentralklinik.de.

RESUMEN / SUMMARY:  - Pancreatic neuroendocrine tumors originate from the diffuse neuroendocrine system in the pancreatic region. These tumors exhibit a rising incidence despite their rareness and due to their benign behavior a considerable prevalence. Pathogenesis of pancreatic neuroendocrine tumors is characterized by common pathways of hereditary and sporadic tumors. Pancreatic neuroendocrine tumors may secrete peptide hormones or biogenic amines in an autonomous fashion as functional active tumors. Pathological grading and staging by TNM systems has been established in recent years classifying well and moderately differentiated pancreatice neuroendocrine tumors and poorly differentiated neuroendocrine carcinomas. Chromogranin A and less so pancreatic polypeptide are suitable tumor markers for  pancreatic neuroendocrine tumors. Expression of receptors for somatostatin is the basis of treatment of pancreatic neuroendocrine tumors with somatostatin analogues as antisecretive and antiproliferative agents. In addition, somatostatin scintigraphy or PET/CT allows comprehensive diagnosis of pancreatic  neuroendocrine tumors, which should be supported by (endoscopic and contrast enhanced) ultrasound, CT and MRI. Therapy of pancreatic neuroendocrine tumors consists of somatostatin analogues, chemotherapy, targeted therapy and peptide receptor radionuclide therapy. Two molecular substances hav been registered for pancreatic neuroendocrine tumors recently, sunitinib (Sutent®) and everolimus (Afinitor®). Predominant tumor load in the liver may be treated by local ablative therapy or liver transplantation. These treatment options have been included in guidelines of several professional societies and weighted for sequential therapy of patients with pancreatic neuroendocrine tumors according to effects and side effects.

 

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[164]

TÍTULO / TITLE:  - Genotype-phenotype associations in neurofibromatosis type 1 (NF1): an increased risk of tumor complications in patients with NF1 splice-site mutations?

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Hum Genomics. 2012 Aug 13;6:12. doi: 10.1186/1479-7364-6-12.

            ●● Enlace al texto completo (gratuito o de pago) 1186/1479-7364-6-12

AUTORES / AUTHORS:  - Alkindy A; Chuzhanova N; Kini U; Cooper DN; Upadhyaya M

INSTITUCIÓN / INSTITUTION:  - Clinical Genetics Department, Sultan Qaboos University Hospital, Al-Khod, Muscat  123, Sultanate of Oman.

RESUMEN / SUMMARY:  - Neurofibromatosis type 1 (NF1) is a complex neurocutaneous disorder with an increased susceptibility to develop both benign and malignant tumors but with a wide spectrum of inter and intrafamilial clinical variability. The establishment  of genotype-phenotype associations in NF1 is potentially useful for targeted therapeutic intervention but has generally been unsuccessful, apart from small subsets of molecularly defined patients. The objective of this study was to evaluate the clinical phenotype associated with the specific types of NF1 mutation in a retrospectively recorded clinical dataset comprising 149 NF1 mutation-known individuals from unrelated families. Each patient was assessed for ten NF1-related clinical features, including the number of cafe-au-lait spots, cutaneous and subcutaneous neurofibromas and the presence/absence of intertriginous skin freckling, Lisch nodules, plexiform and spinal neurofibromas, optic gliomas, other neoplasms (in particular CNS gliomas, malignant peripheral nerve sheath tumors (MPNSTs), juvenile myelomonocytic leukemia, rhabdomyosarcoma, phaechromocytoma, gastrointestinal stromal tumors, juvenile xanthogranuloma, and  lipoma) and evidence of learning difficulties. Gender and age at examination were also recorded. Patients were subcategorized according to their associated NF1 germ line mutations: frame shift deletions (52), splice-site mutations (23), nonsense mutations (36), missense mutations (32) and other types of mutation (6). A significant association was apparent between possession of a splice-site mutation and the presence of brain gliomas and MPNSTs (p = 0.006). If confirmed,  these findings are likely to be clinically important since up to a third of NF1 patients harbor splice-site mutations. A significant influence of gender was also observed on the number of subcutaneous neurofibromas (females, p = 0.009) and preschool learning difficulties (females, p = 0.022).

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[165]

TÍTULO / TITLE:  - Extra Gastrointestinal Stromal Tumor treated with imatinib in a patient with Neurofibromatosis type 1.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Gastrointest Oncol. 2012 Dec;3(4):373-6. doi: 10.3978/j.issn.2078-6891.2012.034.

            ●● Enlace al texto completo (gratuito o de pago) 3978/j.issn.2078-6891.2012.034

AUTORES / AUTHORS:  - Malhotra A; Wright J; Gajra A

INSTITUCIÓN / INSTITUTION:  - SUNY Upstate Medical University Syracuse, New York, USA.

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[166]

TÍTULO / TITLE:  - Merkel cell polyomavirus and trichodysplasia spinulosa-associated polyomavirus DNAs and antibodies in blood among the elderly.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - BMC Infect Dis. 2012 Dec 28;12:383. doi: 10.1186/1471-2334-12-383.

            ●● Enlace al texto completo (gratuito o de pago) 1186/1471-2334-12-383

AUTORES / AUTHORS:  - Sadeghi M; Aronen M; Chen T; Jartti L; Jartti T; Ruuskanen O; Soderlund-Venermo M; Hedman K

INSTITUCIÓN / INSTITUTION:  - Department of Virology, Haartman Institute, University of Helsinki, Helsinki, Finland. reza.sadeghi@helsinki.fi.

RESUMEN / SUMMARY:  - ABSTRACT: BACKGROUND: Merkel cell polyomavirus (MCPyV) and trichodysplasia spinulosa-associated polyomavirus (TSPyV) are recently found pathogens causing two rare skin disorders, Merkel cell carcinoma (MCC) and trichodysplasia spinulosa (TS). MCC is proportionally common in the elderly and most often is associated with immunosuppression. TS is a folliculocentric infection seen in patients in an immunocompromised state. Little or no baseline information exists, however, on the prevalences of these two viruses among the elderly. Epidemiologic data on this population could help in understanding their natural biology. We wished to determine the occurrences and blood levels of MCPyV and TSPyV DNAs among the elderly and any association between the prevalences of their corresponding antiviral IgG antibodies. METHODS: From 394 hospitalized elderly individuals (age >/=65 years) with respiratory symptoms, cardiovascular, and other diseases, we studied 621 serum samples by four different real-time quantitative (q) PCRs, two for the DNAs of MCPyV and two for TSPyV. The IgG antibodies for both viruses among 481 serum samples of 326 subjects were measured with enzyme immunoassays (EIAs), using as antigen recombinant virus-like particles (VLPs). RESULTS: Of the 394 patients, 39 (9.9%) were positive at least  once for MCPyV DNA by the LT PCR, and 33 (8.4%) by the VP1 PCR, while 6 (1.5%) were positive by both PCR assays. In general, the viral DNA copy numbers were low. In sharp contrast, no TSPyV DNA was detectable with qPCRs for the corresponding genomic regions. The IgG seroprevalence of MCPyV was 59.6% and of TSPyV, 67.3%. CONCLUSIONS: MCPyV DNA, unlike TSPyV DNA, occurs in low copy number in serum samples from a notable proportion of aging individuals. Whether this reflects enhanced viral replication possibly due to waning immune surveillance, and is associated with increased MCC risk, deserves exploration.

 

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[167]

TÍTULO / TITLE:  - Incidence and Survival of Patients with Small Intestinal Neuroendocrine Tumours in a Danish NET Center.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - ScientificWorldJournal. 2012;2012:206350. doi: 10.1100/2012/206350. Epub 2012 Nov 28.

            ●● Enlace al texto completo (gratuito o de pago) 1100/2012/206350

AUTORES / AUTHORS:  - Hoej LB; Nykjaer KM; Gronbaek H

INSTITUCIÓN / INSTITUTION:  - Department of Medicine V (Hepatology and Gastroenterology), Aarhus University Hospital, 8000 Aarhus, Denmark.

RESUMEN / SUMMARY:  - Introduction. Small intestinal neuroendocrine tumours (NETs) have increased in incidence during the past decades. In recent years, new promising treatment modalities have been introduced. The aim of the present study was to characterize and compare patients with small intestinal NET seen in the periods 1994-2003 (group 1) and 2004-2011 (group 2) to demonstrate changes in incidence and survival in the two time periods. Patients and Methods. There were 52 NET patients in group 1 and 109 patients in group 2. Results. The incidence of small  intestinal NET was 0.3/100.000/year in period 1 and 0.7/100.000/year in period 2. There was no difference in median chromogranin A levels (8709 versus 2381 pmol/L, P = 0.107), presence of liver metastases (56% versus 44%), clinical symptoms (flushing/diarrhea), or Ki67 index (2% versus 2%), between the two time periods.  The 5-year survival rate in the two time periods was similar, 64.7%, and 77.0%, respectively, (P = 0.23). Conclusion. We observed an increase in the incidence of small intestinal NET during the period from January 1994 to December 2011, but we were not able to demonstrate an improved survival during the same time period.

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[168]

TÍTULO / TITLE:  - (68)Ga-DotaTATE PET-CT followed by Peptide Receptor Radiotherapy in combination with capecitabine in two patients with Merkel Cell Carcinoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Int J Clin Exp Med. 2012;5(4):363-6. Epub 2012 Sep 28.

AUTORES / AUTHORS:  - Schmidt MC; Uhrhan K; Markiefka B; Hasselbring L; Schlaak M; Cremer B; Kunze S; Baum RP; Dietlein M

INSTITUCIÓN / INSTITUTION:  - Department of Nuclear Medicine, University Hospital of Cologne Germany.

RESUMEN / SUMMARY:  - Herein, we report about two Caucasian patients with the histopathological diagnosis of Merkel cell carcinoma suffering from extensive lymph node metastases. The extent of the disease was diagnosed by Ga-68-DotaTATE-PET-CT. Both patients had rapid disease progression, one of them despite a three months course of sunitinibe followed by four chemotherapy cycles of cisplatin and etoposide. Both patients were sent for peptide receptor radiotherapy with 90Y-DotaTATE or 177Lu-DotaTATE in combination with capecitabine. Additional external beam radiotherapy of the cervical and inguinal lymph nodes was given to  the patient with progressive disease despite chemotherapy. Temporary partial response in both patients was achieved. Despite extensive therapeutic efforts, fatal outcome could not be prevented 10 and 14 months after first clinical symptoms.

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[169]

TÍTULO / TITLE:  - Long-term disease control of a non-operable neuroendocrine tumor of the lung with lanreotide: a case study.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Case Rep Oncol. 2012 Sep;5(3):657-66. doi: 10.1159/000345695. Epub 2012 Dec 18.

            ●● Enlace al texto completo (gratuito o de pago) 1159/000345695

AUTORES / AUTHORS:  - Van Fraeyenhove F; De Droogh E; Meireson N; Galdermans D; Goor C; Van Acker F; Mattelaer C; De Ruyter V; Schrijvers D

INSTITUCIÓN / INSTITUTION:  - Department of Medical Oncology, Ziekenhuisnetwerk Antwerpen, Antwerp, Belgium.

RESUMEN / SUMMARY:  - Bronchopulmonary neuroendocrine tumors (NETs) are malignant tumors that represent approximately 20% of all lung cancers. The therapeutic option for advanced or metastatic bronchopulmonary NETs is mainly palliation of symptoms; options need to be individualized and, therefore, rely on the knowledge of multidisciplinary teams. Somatostatin analogs have been widely used in NETs for control of hormonal syndromes and are currently under evaluation for their antiproliferative activity. Here, we present a case of NET of the lung, for which we achieved long-term disease control with a treatment comprising the somatostatin analog lanreotide Autogel(®) in a patient with limited therapeutic options due to considerable comorbidity, while preserving his quality of life.

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[170]

TÍTULO / TITLE:  - Combination Chemotherapy with Cyclophosphamide, Vincristine, and Dacarbazine in Patients with Malignant Pheochromocytoma and Paraganglioma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Horm Cancer. 2013 Jan 30.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s12672-013-0133-2

AUTORES / AUTHORS:  - Tanabe A; Naruse M; Nomura K; Tsuiki M; Tsumagari A; Ichihara A

INSTITUCIÓN / INSTITUTION:  - Department of Medicine II, Tokyo Women’s Medical University, 8-1, Kawadacho, Shinjuku-ku, Tokyo, 162-8666, Japan, akiyotana@endm.twmu.ac.jp.

RESUMEN / SUMMARY:  - Choosing effective therapy for patients with malignant pheochromocytoma or paraganglioma (PPGL) is problematic and none of the options are curative. Although combination chemotherapy with cyclophosphamide, vincristine, and dacarbazine (CVD) is an established treatment option, only a limited number of case series have been reported in the literature. To determine the efficacy of CVD in patients treated at Tokyo Women’s Medical University. Retrospective review of patients treated with CVD between 1989 and 2012 was conducted. Demographics, clinical presentation, imaging, and laboratory reports were reviewed and analyzed. Efficacy of CVD was ascertained from the biochemical and tumor responses. Twenty-three patients fulfilled study criteria and 6 of these were excluded due to inadequate follow-up or discontinuance by poor general condition  or adverse effects. Thus, 17 cases were included in the study. The age and duration of the disease before initiation of CVD were 54.7 +/- 12.0 years and 9.1 +/- 8.1 years, respectively. The follow-up period after initiation of CVD ranged  from 12 to 192 months (median, 60 months). Complete or partial biochemical and/or partial tumor response was achieved in 47.1 % (responders). No significant biochemical or tumor response was seen in 23.5 % and deterioration in biochemical and tumor outcomes was seen in 29.4 % (non-responders). No patient showed complete biochemical and tumor responses. In responders, these effects were documented within 4 months after initiation of CVD with a progression-free survival of 31 to 60 months (median, 40 months). Age at the first diagnosis with  PPGL was younger (P < 0.05) and the lag time to eventual diagnosis of malignant disease was longer (P < 0.05) in responders than those in non-responders. The responders had improvements in hypertension and impaired glucose tolerance. Although CVD chemotherapy is not curative for patients with malignant PPGL, it does provide approximately half of the patients with biochemical, tumor, and hypertension benefits.

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[171]

TÍTULO / TITLE:  - Metastatic Merkel cell carcinoma of the oral cavity in a human immunodeficiency virus-positive patient and the detection of Merkel cell polyomavirus.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Oral Surg Oral Med Oral Pathol Oral Radiol. 2012 Dec 11. pii: S2212-4403(12)01510-6. doi: 10.1016/j.oooo.2012.09.002.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.oooo.2012.09.002

AUTORES / AUTHORS:  - Li M; Saghafi N; Freymiller E; Basile JR; Lin YL

INSTITUCIÓN / INSTITUTION:  - Division of Diagnostic and Surgical Sciences, School of Dentistry, University of  California, Los Angeles, California.

RESUMEN / SUMMARY:  - The etiology of Merkel cell carcinoma (MCC) was recently linked to a newly identified human virus, the Merkel cell polyomavirus (MCPyV). The discovery that  MCPyV plays an important role in the tumorigenesis of >80% of MCCs provides an explanation for the increased incidence of this rare malignancy in human immunodeficiency virus (HIV)-positive and immunocompromised patients. We report an unusual metastasis of MCC to the mandibular gingiva of an HIV-positive patient. In addition to routine hematoxylin-eosin and immunohistochemical studies, we also performed a molecular biologic analysis to look for the presence of MCPyV in this case. We detected evidence of the MCPyV genome in this lesion similar to what has been observed for MCCs reported in other immunocompromised patients. These results stress the importance of combining morphologic and molecular biologic analyses in the evaluation of MCC, because confirmation of viral etiology would likely affect the choice of treatment and prognosis when specific antiviral therapy becomes available for this aggressive tumor.

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[172]

TÍTULO / TITLE:  - Safety and efficacy of sunitinib in patients with unresectable pancreatic neuroendocrine tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Clin Med Insights Oncol. 2012;6:381-93. doi: 10.4137/CMO.S7350. Epub 2012 Nov 20.

            ●● Enlace al texto completo (gratuito o de pago) 4137/CMO.S7350

AUTORES / AUTHORS:  - Wiedmann MW; Mossner J

INSTITUCIÓN / INSTITUTION:  - Department of Internal Medicine I, St. Mary’s Hospital, Berlin, Germany. ; Division of Gastroenterology and Rheumatology, Department of Medicine, Neurology  and Dermatology, University Hospital of Leipzig, Leipzig, Germany.

RESUMEN / SUMMARY:  - Pancreatic neuroendocrine tumors (PNETs) are becoming increasingly common, with the majority of patients presenting with either lymph node involvement or metastatic disease, thus requiring systemic therapy. Targeted therapy is a type of medication that blocks the growth of cancer cells by interfering with specific targeted molecules needed for carcinogenesis and tumor growth rather than by simply interfering with rapidly dividing cells (eg, with traditional chemotherapy). In this review article, pharmacologic inhibition of multiple targets including vascular endothelial growth factor receptor (VEGF-R), platelet-derived growth factor receptor (PDGF-R), stem cell factor receptor (c-KIT-R), FML-like tyrosine kinase-3 receptor (FLT3-R), colony stimulating factor 1 receptor (CSF1-R), and glial cell-line derived neurotrophic factor receptor (RET-R) with sunitinib in patients with unresectable PNETs is discussed. Phase III data indicate that additional treatment with sunitinib can improve prognosis in these patients.

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[173]

TÍTULO / TITLE:  - Mucinous cystic neoplasm of the pancreas with neuroendocrine cells and malignant  stroma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Saudi Med J. 2013 Jan;34(1):80-5.

AUTORES / AUTHORS:  - Asberry DE; Youngberg GA; Al-Abbadi MA

INSTITUCIÓN / INSTITUTION:  - Department of Pathology and Laboratory Medicine, James H. Quillen VA Medical Center, East Tennessee State University College of Medicine, Johnson City, Tennessee, United States of America.

RESUMEN / SUMMARY:  - Mucinous cystic neoplasms (MCN) with malignant sarcomatous stroma are rare aggressive tumors and there are few recorded cases. We report a case of MCN that  had adenocarcinoma in situ and invasive adenocarcinoma with foci of sarcomatous stroma in a 40-year-old woman. Clear transition from adenocarcinoma areas into sarcomatoid foci was noted. The stromal component showed immunoreactivity for CK7 and Cam 5.2 supporting epithelial origin of the sarcomatoid areas. Associated areas of cytologically benign MCN epithelium were present and were immunoreactive for positive staining with pan-cytokeratin (AE1/AE3), cytokeratin 7 (CK 7), cytokeratin 20 (CK 20), pan-cytokeratin (Cam 5.2), epithelial membrane antigen (EMA), muscle specific actin (MSA), and carcino-embryonic antigen (CEA). Interestingly, definite scattered pear-shaped neuroendocrine cells, as evidenced  by strong immunoreactivity for chromogranin and synaptophysin, were identified in the cytologically benign MCN lining but not in the malignant epithelial component. We found that these tumor cells probably arise from a single precursor cell capable of divergent differentiation.

 

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[174]

TÍTULO / TITLE:  - Experience of significant hemodynamic instability that occurred during excisional biopsy in a patient with unrecognized bronchial carcinoid tumor.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Korean J Anesthesiol. 2012 Nov;63(5):475-6. doi: 10.4097/kjae.2012.63.5.475. Epub 2012 Nov 16.

            ●● Enlace al texto completo (gratuito o de pago) 4097/kjae.2012.63.5.475

AUTORES / AUTHORS:  - Lee S; Lee KH; Kim JY; Choe WJ; Kim JW

INSTITUCIÓN / INSTITUTION:  - Department of Anesthesiology and Pain Medicine, Ilsan Paik Hospital, Inje University School of Medicine, Goyang, Korea.

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[175]

TÍTULO / TITLE:  - Microvascular density and mast cells in benign and malignant pheochromocytomas.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Pol J Pathol. 2012 Dec;63(4):235-42.

AUTORES / AUTHORS:  - Bialas M; Dyduch G; Szpor J; Demczuk S; Okon K

INSTITUCIÓN / INSTITUTION:  - Magdalena Bialas MD, Department of Pathomorphology, ul. Grzegorzecka 16, 31-351 Krakow, tel. +48 12 421 15 64, e-mail: mbialas7@gmail.com.

RESUMEN / SUMMARY:  - Pheochromocytomas, uncommon adrenal tumors, have an uncertain behavior. Recently, PASS criteria were proposed for differentiating between benign and malignant cases. These are not perfect, however. The aim of the study was to investigate angiogenesis and mast cell density in context of the clinical behavior and morphologic characteristics of pheochromocytomas. Mean intratumoral chymase positive cell count was 14.50 for malignant, 15.73 for benign cases; mean subcapsular chymase positive cell count was 12.50 for malignant, 11.27 for benign cases. Mean intratumoral tryptase positive cell count was 17.50 for malignant and 17.91 for benign cases; mean subcapsular tryptase positive cell count was 15.25 for malignant and 15.73 for benign cases. Mean intratumoral CD31 positive vessel  count was 46.98 for malignant and 51.02 for benign cases; mean subcapsular CD31 positive vessel count was 44.86 for malignant and 39.81 for benign cases. Mean intratumoral CD105 positive vessel count was 37.84 for malignant and 35.95 for benign cases; mean subcapsular CD105 positive vessel count was 26.36 for malignant and 22.03 for benign cases. The differences between benign and malignant cases were not significant. All the vascular counts were correlated with mast cells counts. PASS index was inversely correlated with mast cell counts.

 

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[176]

TÍTULO / TITLE:  - Goblet cell carcinoid in a patient with neurofibromatosis type 1: a rare combination.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Case Rep Gastrointest Med. 2012;2012:185730. doi: 10.1155/2012/185730. Epub 2012  Dec 4.

            ●● Enlace al texto completo (gratuito o de pago) 1155/2012/185730

AUTORES / AUTHORS:  - Gregersen T; Holt N; Gronbaek H; Vogel I; Jorgensen LJ; Krogh K

INSTITUCIÓN / INSTITUTION:  - Department of Medicine, Hepatology and Gastroenterology, Aarhus University Hospital, Norrebrogade 44, 8000 Aarhus C, Denmark.

RESUMEN / SUMMARY:  - Neuroendocrine tumors are rare tumors primarily located in the gastrointestinal tract. Goblet cell carcinoid is a rare subgroup of neuroendocrine tumors located  in the appendix. Neurofibromatosis type 1 is an autosomal dominant disorder caused by a mutation in the NF1 gene. Patients with neurofibromatosis type 1 have an increased incidence of typical neuroendocrine tumors, but it is unknown if this is the case with goblet cell carcinoids. We describe a patient with both neurofibromatosis type 1 and goblet cell carcinoid, that according to literature  would occur in 0.00017 per million per year. This may suggest a previously unknown association between neurofibromatosis type 1 and goblet cell carcinoids.

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[177]

TÍTULO / TITLE:  - Recurrent acute pancreatitis in a patient with wirsungocele and neuroendocrine tumor of ampulla of vater.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - JOP. 2013 Jan 10;14(1):99-101. doi: 10.6092/1590-8577/1310.

AUTORES / AUTHORS:  - Dhir V; Joshi N; Vivekanandarajah S; Bhandari S; Bapat M; Maydeo A

INSTITUCIÓN / INSTITUTION:  - Baldota Institute of Digestive Sciences. Mumbai, India. vinaydhir@gmail.com.

RESUMEN / SUMMARY:  - CONTEXT: Wirsungocele has recently been shown to be associated with acute recurrent, severe necrotizing pancreatitis and chronic pancreatitis or chronic pain in abdomen. Till to date there is no report on association of wirsungocele with an ampullary neuroendocrine tumor, and recurrent pancreatitis. CASE REPORT:  We report a first ever case of wirsungocele diagnosed on EUS, its association with neuroendocrine tumor of ampulla and recurrent acute pancreatitis. CONCLUSION: This case report highlights the diagnostic utility of EUS in diagnosing small ampullary pathology like wirsungocele and neuroendocrine tumor.

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[178]

TÍTULO / TITLE:  - Pulmonary carcinoid tumorlet without underlying lung disease: analysis of its relationship to fibrosis.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Thorac Dis. 2012 Dec;4(6):655-8. doi: 10.3978/j.issn.2072-1439.2012.06.11.

            ●● Enlace al texto completo (gratuito o de pago) 3978/j.issn.2072-1439.2012.06.11

AUTORES / AUTHORS:  - He P; Gu X; Wu Q; Lin Y; Gu Y; He J

INSTITUCIÓN / INSTITUTION:  - Department of Pathology, The First Affiliated Hospital of Guangzhou Medical College, Guangzhou 510120, China;

RESUMEN / SUMMARY:  - Pulmonary carcinoid tumorlet is a rare pathology and appears to be always associated with other lesions such as bronchiectasis and fibrosis. While the caus e-effect relationship between tumorlet and the accompanying pathological changes  in the surrounding mesenchymal tissue remains to be defined, it has been postulated that pulmonary fibrosis may be the primary pathology underlying the development of tumorlet. In this paper, we present a case where a tumor (<0.5 cm) was detected in the right upper lobe of a 71-year old woman. Cells of the tumor displayed markers characterizing for their neuroendocrine origin. No histological evidence for inflammation, interstitial fibrosis and remodeling of vascular structure was observed. However, immunohistochemistry assay demonstrated a strong production of the profibrotic factors VEGF and TGF-beta1 by tumor cells. These findings suggest that carcinoid tumorlet can be an isolated lesion and pulmonary  fibrosis that “often co-exists” with tumorlet may be secondary to the paracrine effects of fibrotic growth factors produced by tumorlet.

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[179]

TÍTULO / TITLE:  - Clear cell neuroendocrine tumor G1 of the gallbladder without von Hippel-Lindau disease.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Oncol Lett. 2012 Dec;4(6):1174-1176. Epub 2012 Sep 6.

            ●● Enlace al texto completo (gratuito o de pago) 3892/ol.2012.899

AUTORES / AUTHORS:  - Ishida M; Shiomi H; Naka S; Tani T; Okabe H

INSTITUCIÓN / INSTITUTION:  - Department of Clinical Laboratory Medicine and Division of Diagnostic Pathology;

RESUMEN / SUMMARY:  - Clear cell neuroendocrine tumor (NET) is a rare but distinct histopathological variant of NET most often observed in patients with von Hippel-Lindau disease (VHL). Clear cell NET in non-VHL patients is extremely rare and this report is only the second to describe a case of clear cell NET G1 (carcinoid tumor) of the  gallbladder without VHL. A 71-year-old male without past or family history of VHL presented with a polypoid lesion in the fundus of the gallbladder and laparoscopic cholecystectomy was performed. Histopathological study revealed that the polypoid lesion was comprised of nests or trabecular growths of clear cells without atypia. Immunohistochemically, the neoplastic cells were diffusely positive for endocrine markers, but negative for alpha-inhibin. Clinicopathological review of cases with clear cell NET G1 of the gallbladder revealed that this disease occurs in patients with or without VHL, but that gallbladder stones and cholecystitis were present in non-VHL cases. We hypothesized that the occurrence of clear cell NET of the gallbladder (particularly non-VHL cases) may be associated with chronic cholecystitis induced by gallbladder stones. Moreover, alpha-inhibin was detected in clear cell NET tumor cells in one VHL case, but not in two non-VHL cases. These findings suggest that alpha-inhibin expression is a useful determinant of an association between clear cell NET and VHL.

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[180]

TÍTULO / TITLE:  - Plexiform (multinodular) schwannoma of soft palate. Report of a case.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Folia Med (Plovdiv). 2012 Jul-Sep;54(3):62-4.

AUTORES / AUTHORS:  - Kapetanakis S; Vasileiadis I; Petousis A; Fiska A; Stavrianaki A

INSTITUCIÓN / INSTITUTION:  - Department of Anatomy, Medical school of Alexandroupolis, Democritus University of Thrace, Heraklion, Greece. kastegepe@yahoo.gr

RESUMEN / SUMMARY:  - Plexiform schwannoma is a rare benign neoplasm of the neural sheath characterized by a multinodular plexiform growth pattern. Only 5% of schwannomas have a plexiform or multinodular growth pattern. Schwannoma apparently derives from the  Schwann cells. Extracranially, 25% of all schwannomas are located in the head and neck region, but only 1% show an intraoral origin. The intraoral lesions show a predilection for the tongue, followed by the palate, buccal mucosa, lip and gingival. Microscopic examination is necessary to confirm the diagnosis. Characteristic histological signs are the palisading of the spindle-shaped Schwann cells around the central acellular area, so called Verocay bodies. We report a case of a 21-year-old woman with a smooth mass of the soft palate that was gradually increasing. Surgical excision of the mass was done and the histopathology and immunohistochemistry study of the excised lesion revealed a multinodular plexiform schwannoma of the soft palate. The patient is under regular clinical control, with no signs of recurrence after 17 months. Plexiform  schwannomas of the soft palate are mentioned very rarely in the English literature. This rare benign tumor is worthy of recognition because it can be misdiagnosed as plexiform neurofibroma.

 

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[181]

TÍTULO / TITLE:  - Selected neuroendocrine tumour markers, growth factors and their receptors in typical and atypical bronchopulmonary carcinoids.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endokrynol Pol. 2012;63(6):477-82.

AUTORES / AUTHORS:  - Telega A; Kos-Kudla B; Foltyn W; Blicharz-Dorniak J; Rosiek V

RESUMEN / SUMMARY:  - Introduction: Bronchopulmonary neuroendocrine tumours (BP NET) cause many diagnostic and therapeutic problems. There is an ongoing search for biochemical markers of activity of these tumours. The use of polypeptide growth factors seems potentially feasible in establishing the diagnosis, prognosis and treatment of these tumours. Material and methods: We included 41 patients aged 25 to 78 years  with histopathologically confirmed typical and atypical bronchopulmonary carcinoid tumours and 20 healthy volunteers. We assessed the levels of specific and non-specific markers of these tumours and of selected growth factors relative to TNM classification. Results: The levels of specific markers (serotonin and its metabolite, 5-hydroxyindoleacetic acid [5HIAA]) and non-specific markers (chromogranin A [CgA]) were significantly higher in patients with atypical carcinoid tumours. The serum levels of hepatocyte growth factor (HGF), vascular endothelial growth factor (VEGF) and VEGF receptor-1 (VEGFR-1) were significantly higher in patients with carcinoid tumours versus the control group. The levels of VEGFR-1 closely correlated with TNM classification. No such correlation could, however, be confirmed for the levels of HGF, VEGF or VEGFR-2. Conclusions: Determination of CgA, serotonin and 5HIAA may be useful in the diagnosis of BP NET, particularly in atypical carcinoid tumours, and their levels depend on the presence of distant metastases. Determination of growth factors (VEGF and its receptor, VEGFR1, and HGF) may prove useful in the clinical diagnosis of these tumours, while the assessment of VEGFR1 expression may be helpful in tumour staging. (Endokrynol Pol 2012; 63 (6): 477-482).

 

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[182]

TÍTULO / TITLE:  - Group VIB Phospholipase A(2) promotes proliferation of INS-1 insulinoma cells and attenuates lipid peroxidation and apoptosis induced by inflammatory cytokines and oxidant agents.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Oxid Med Cell Longev. 2012;2012:989372. doi: 10.1155/2012/989372. Epub 2012 Nov 11.

            ●● Enlace al texto completo (gratuito o de pago) 1155/2012/989372

AUTORES / AUTHORS:  - Bao S; Song H; Tan M; Wohltmann M; Ladenson JH; Turk J

INSTITUCIÓN / INSTITUTION:  - Mass Spectrometry Resource, Division of Endocrinology, Metabolism, and Lipid Research, Department of Medicine, Washington University School of Medicine, Campus Box 8127, 660 S. Euclid Avenue, St. Louis, MO 63110, USA.

RESUMEN / SUMMARY:  - Group VIB Phospholipase A(2) (iPLA(2)gamma) is distributed in membranous organelles in which beta-oxidation occurs, that is, mitochondria and peroxisomes, and is expressed by insulin-secreting pancreatic islet beta-cells and INS-1 insulinoma cells, which can be injured by inflammatory cytokines, for example, IL-1beta and IFN-gamma, and by oxidants, for example, streptozotocin (STZ) or t-butyl-hydroperoxide (TBHP), via processes pertinent to mechanisms of beta-cell  loss in types 1 and 2 diabetes mellitus. We find that incubating INS-1 cells with IL-1beta and IFN-gamma, with STZ, or with TBHP causes increased expression of iPLA(2)gamma mRNA and protein. We prepared INS-1 knockdown (KD) cell lines with reduced iPLA(2)gamma expression, and they proliferate more slowly than control INS-1 cells and undergo increased membrane peroxidation in response to cytokines  or oxidants. Accumulation of oxidized phospholipid molecular species in STZ-treated INS-1 cells was demonstrated by LC/MS/MS scanning, and the levels in  iPLA(2)gamma-KD cells exceeded those in control cells. iPLA(2)gamma-KD INS-1 cells also exhibited higher levels of apoptosis than control cells when incubated with STZ or with IL-1beta and IFN-gamma. These findings suggest that iPLA(2)gamma promotes beta-cell proliferation and that its expression is increased during inflammation or oxidative stress as a mechanism to mitigate membrane injury that  may enhance beta-cell survival.

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[183]

TÍTULO / TITLE:  - Genetic studies in a coexistence of acromegaly, pheochromocytoma, gastrointestinal stromal tumor (GIST) and thyroid follicular adenoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Arq Bras Endocrinol Metabol. 2012 Nov;56(8):507-12.

AUTORES / AUTHORS:  - Boguszewski CL; Fighera TM; Bornschein A; Marques FM; Denes J; Rattenbery E; Maher ER; Stals K; Ellard S; Korbonits M

INSTITUCIÓN / INSTITUTION:  - Endocrine Division, Department of Internal Medicine, University Hospital at Universidade Federal do Parana, Curitiba, Brazil.

RESUMEN / SUMMARY:  - We report on an adult woman with rare coexistence of acromegaly, pheochromocytoma (PHEO), gastrointestinal stromal tumor (GIST), intestinal polyposis, and thyroid  follicular adenoma. At the age of 56, she was diagnosed with acromegaly caused by a pituitary macroadenoma, treated by transsphenoidal surgery, radiotherapy, and octreotide. During routine colonoscopy, multiple polyps were identified as tubular adenomas with high-grade dysplasia on histology. Years later, an abdominal mass of 8.0 x 6.2 cm was detected by routine ultrasound. Surgical exploration revealed an adrenal mass and another tumor adhered to the lesser gastric curvature, which were removed. Pathology confirmed the diagnosis of PHEO  and GIST. PHEO immunohistochemistry was negative for GHRH. During follow-up, nodular goiter was found with normal levels of calcitonin and inconclusive cytology. Near-total thyroidectomy was performed, revealing a follicular adenoma. Her family history was negative for all of these tumor types. Genetic analysis for PHEO/paraganglioma genes (SDH A-D, SDHAF2, RET, VHL, TMEM127, and MAX), and pituitary-related genes (AIP, MEN1, and p27) were negative. Though the finding of PHEO and acromegaly with multiple other tumors could be a fortuitous coexistence, we suggest that this case may represent a new variant of MEN syndrome with a de novo germline mutation in a not yet identified gene. Arq Bras Endocrinol Metab. 2012;56(8):507-12.

 

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[184]

TÍTULO / TITLE:  - A case of gastric neuroendocrine carcinoma with liver metastasis and portal vein  invasion successfully treated by S-1 and cisplatin chemotherapy.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Nihon Shokakibyo Gakkai Zasshi. 2013;110(1):56-63.

AUTORES / AUTHORS:  - Kaneko S; Itsui Y; Hashiguchi M; Watanabe M; Yoshida R; Akashi T; Yauchi T; Watanabe M

INSTITUCIÓN / INSTITUTION:  - Department of Gastroenterology, Soka Municipal Hospital.

RESUMEN / SUMMARY:  - A 66-year-old man presented with anemia. Endoscopy revealed an elevated lesion with ulceration in the posterior wall of the lesser curvature of the lower gastric body. Endoscopic biopsy demonstrated pathological diagnosis, neuroendocrine carcinoma. A computed tomography scan showed liver metastasis and  portal vein invasion. We started chemotherapy with S-1 and cisplatin. After six courses of treatment over seven months, partial response was assessed. We are continuing this chemotherapy now.

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[185]

TÍTULO / TITLE:  - A Case of Medullary Thyroid Carcinoma with de novo V804M RET Germline Mutation.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Korean Med Sci. 2013 Jan;28(1):156-9. doi: 10.3346/jkms.2013.28.1.156. Epub 2013 Jan 8.

            ●● Enlace al texto completo (gratuito o de pago) 3346/jkms.2013.28.1.156

AUTORES / AUTHORS:  - Choi YS; Kwon HJ; Kim BK; Kwon SK; Park YH; Kim JH; Jung SB; Lee CH; Lee SK; Uchino S

INSTITUCIÓN / INSTITUTION:  - Department of Internal Medicine, Kosin University College of Medicine, Busan, Korea.

RESUMEN / SUMMARY:  - Many cases of RET proto-oncogene mutations of hereditary medullary thyroid carcinoma (MTC) have been reported in Korea. However, MTC with V804M RET proto-oncogene germline mutations have not been reported in Korea. A 33-yr-old man was diagnosed with a 0.7-cm sized thyroid nodule. Laboratory testing revealed serum calcitonin was elevated. The patient underwent total thyroidectomy with central compartment neck dissection for the thyroid tumor. RET gene analysis was  performed in both the index patient and his family. There were no V804M RET mutation and abnormal laboratory findings within his family except the index patient. Therefore, this patient was a de novo V804M RET germline mutation.

 

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[186]

TÍTULO / TITLE:  - Medical treatment of neuroendocrine tumours.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Curr Opin Endocrinol Diabetes Obes. 2013 Feb;20(1):27-31. doi: 10.1097/MED.0b013e32835c034f.

            ●● Enlace al texto completo (gratuito o de pago) 1097/MED.0b013e32835c034f

AUTORES / AUTHORS:  - Weber HC

INSTITUCIÓN / INSTITUTION:  - Boston University School of Medicine, Section of Gastroenterology, Boston, Massachusetts, USA. christian.weber@bmc.org

RESUMEN / SUMMARY:  - PURPOSE OF REVIEW: This review summarizes results of recent clinical trials regarding the treatment of advanced neuroendocrine tumours (NETs) and pancreatic  NETs (PNETs). RECENT FINDINGS: Most NETs occur sporadically in the lung and the gastrointestinal tract, and their prevalence has apparently increased over the last decades. Although curative treatment can be accomplished by surgery, for some NETs, most present in advanced stages and alternative, medical therapy is indicated. Recent randomized clinical treatment trials using somatostatin analogues in well differentiated midgut NET and therapies targeting the mammalian target of rapamycin (mTOR) signalling pathway and various tyrosine kinases provided evidence of improved progression-free survival. Treatment of functional  PNETs with the mTOR inhibitor everolimus also showed reduction of peptide secretion relevant to the presenting clinical syndrome. SUMMARY: Previous work regarding the molecular pathology of NETs identified mTOR and tyrosine kinase signalling pathways as relevant targets in the neuroendocrine tumour biology. Subsequently, recent randomized clinical trials targeting these pathways with inhibitor therapies have provided encouraging results demonstrating prolonged progression-free survival and improvement of secretion-related clinical syndromes.

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[187]

TÍTULO / TITLE:  - Genetic and clinical features of multiple endocrine neoplasia types 1 and 2.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Oncol. 2012;2012:705036. doi: 10.1155/2012/705036. Epub 2012 Nov 8.

            ●● Enlace al texto completo (gratuito o de pago) 1155/2012/705036

AUTORES / AUTHORS:  - Romei C; Pardi E; Cetani F; Elisei R

INSTITUCIÓN / INSTITUTION:  - Department of Endocrinology and Metabolism, University of Pisa, 56124 Pisa, Italy.

RESUMEN / SUMMARY:  - Multiple endocrine neoplasia (MEN) are clinical inherited syndromes affecting different endocrine glands. Three different patterns of MEN syndromes can occur (MEN 1, MEN 2A, and MEN 2B). MEN syndromes are very rare, affect all ages and both sexes are equally affected. MEN 1 is characterized by the neoplastic transformation of the parathyroid glands, pancreatic islets, anterior pituitary,  and gastrointestinal tract. Heterozygous MEN 1 germline mutations have been detected in about 70-80% of patients with MEN 1. The mutations are scattered throughout the entire genomic sequence of the gene. MEN 1 patients are characterized by variable clinical features, thus suggesting the lack of a genotype-phenotype correlation. Therapeutical approaches are different according  to the different endocrinopathies. The prognosis is generally good if adequate treatment is provided. In MEN 2 syndromes, the medullary thyroid cancer (MTC) is  almost invariably present and can be associated with pheochromocytoma (PHEO) and/or multiple adenomatosis of parathyroid glands with hyperparathyroidism (PHPT). The different combination of the endocrine neoplasia gives origin to 3 syndromes: MEN 2A, MEN 2B, and FMTC. The clinical course of MTC varies considerably in the three syndromes. It is very aggressive in MEN 2B, almost indolent in the majority of patients with FMTC and with variable degrees of aggressiveness in patients with MEN 2A. Activating germline point mutations of the RET protooncogene are present in 98% of MEN 2 families. A strong genotype-phenotype correlation has been observed and a specific RET mutation may  be responsible for a more or less aggressive clinical course. The treatment of choice for primary MTC is total thyroidectomy with central neck lymph nodes dissection. Nevertheless, 30% of MTC patients, especially in MEN 2B and 2A, are not cured by surgery. Recently, developed molecular therapeutics that target the  RET pathway have shown very promising activity in clinical trials of patients with advanced MTC. MEN 2 prognosis is strictly dependent on the MTC aggressiveness and thus on the success of the initial treatment.

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[188]

TÍTULO / TITLE:  - Exposure to bisphenol A induces dysfunction of insulin secretion and apoptosis through the damage of mitochondria in rat insulinoma (INS-1) cells.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Cell Death Dis. 2013 Jan 17;4:e460. doi: 10.1038/cddis.2012.206.

            ●● Enlace al texto completo (gratuito o de pago) 1038/cddis.2012.206

AUTORES / AUTHORS:  - Lin Y; Sun X; Qiu L; Wei J; Huang Q; Fang C; Ye T; Kang M; Shen H; Dong S

INSTITUCIÓN / INSTITUTION:  - Key Lab of Urban Environment and Health, Department of Environmental and Molecular Toxicology, Institute of Urban Environment, Chinese Academy of Sciences, Xiamen 361021, China.

RESUMEN / SUMMARY:  - Bisphenol A (BPA) is widely used in plastic products, through which humans are exposed to it. Accumulating evidence suggests that BPA exposure is associated with beta-cell dysfunction. Mitochondrial defects can cause impairment and failure of beta cells, but there is little information about the effects of BPA on the mitochondrial function of beta cells. In this study, we assessed the role  of mitochondria-mediated mechanisms underlying BPA-induced beta-cell dysfunction  and resulting beta-cell apoptosis. INS-1 cells were cultured with 0, 0.0020, 0.020, 0.20, or 2.0 muM BPA. Cell viability, glucose-stimulated insulin secretion (GSIS), and mitochondrial function were examined. The mitochondrial apoptotic pathway was also analyzed at molecular level. We found that BPA suppressed cell viability and disturbed GSIS in a dose-dependent manner. Positive Annexin- propidium iodide (PI) staining and altered expression of Bcl-2 family members and caspases in INS-1 cells indicated that the cells progressively became apoptotic after BPA exposure. Additionally, BPA-induced apoptosis was associated with mitochondrial defects in beta cells, as evidenced by depletion of ATP, release of cytochrome c, loss of mitochondrial mass and membrane potential, and alterations  in expression of genes involved in mitochondrial function and metabolism. Taken together, these findings provide strong evidence that BPA triggers INS-1 cells dysfunction and apoptosis may be meditated via the mitochondrial pathway.

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[189]

TÍTULO / TITLE:  - Relationship between clinical characteristics and survival of gastroenteropancreatic neuroendocrine neoplasms: A single-institution analysis (1995-2012) in South China.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - BMC Endocr Disord. 2012 Nov 29;12:30. doi: 10.1186/1472-6823-12-30.

            ●● Enlace al texto completo (gratuito o de pago) 1186/1472-6823-12-30

AUTORES / AUTHORS:  - Wang YH; Lin Y; Xue L; Wang JH; Chen MH; Chen J

INSTITUCIÓN / INSTITUTION:  - Department of Gastroenterology, The First Affiliated Hospital of Sun Yat-sen University, 58 Zhongshan II Road, Guangzhou, People’s Republic of China. chenminhu@vip.163.com.

RESUMEN / SUMMARY:  - ABSTRACT: BACKGROUND: Gastroenteropancreatic neuroendocrine neoplasm (GEP-NEN) is the most common type of neuroendocrine tumors accounting for 65-75% of neuroendocrine neoplasms (NENs). Given the fact that there are few studies on GEP-NENs among Chinese patients, we performed a retrospective study in South China. METHODS: Totally 178 patients with GEP-NENs treated at the First Affiliated Hospital of Sun Yat-sen University between January 1995 and May 2012 were analyzed retrospectively. RESULTS: Pancreas was found the most common site of involvement (34.8%). 149 patients (83.7%) presented as non-functional tumors with non-specific symptoms such as abdominal pain (33.7%); carcinoid syndrome was not found in this study. Several methods are useful for localization of GEP-NENs, yielding varied detection rates from 77.8% to 98.7%. Positive rates of chromogranin A (CgA) and synaptophysin (Syn) immunhistochemically were 69.1% and  90.2%, respectively. 87 patients (51.5%) had G1 tumors, 31(18.3%) G2 tumors and 51 (30.2%) G3 tumors. Neuroendocrine tumor (NET), neuroendocrine carcinoma (NEC)  and mixed adenoendocrine carcinoma (MANEC) were 69.8%, 27.2% and 3.0%, respectively. 28.1% of patients presented with distant disease. Surgery was performed in 152 (85.4%) patients, and overall 5-year survival rate was 54.5%. Functionality, G1 grading and NET classification were associated with favorable prognosis in univariate analysis. Distant metastasis contributed to unfavorable prognosis of these tumors. CONCLUSIONS: Nonfunctional tumors with non-specific symptoms account for the majority of GEP-NENs. Diagnosis depends on pathological  classification. Multidisciplinary treatments could help improve the outcome.

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[190]

TÍTULO / TITLE:  - Goblet cell carcinoid of the vermiform appendix - a case report.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Pol J Pathol. 2012 Dec;63(4):294-7.

AUTORES / AUTHORS:  - Ptaszynski K; Bartczak A; Kolodziej B; Szczepkowski M

INSTITUCIÓN / INSTITUTION:  - Konrad Ptaszynski, Department of Pathology, Medical Centre for Postgraduate Education and Bielanski Hospital, ul. Marymoncka 99, 02-813Warsaw, e-mail: konrad.ptaszynski@cmkp.edu.pl.

RESUMEN / SUMMARY:  - Goblet cell carcinoid (GCC) is a rare neuroendocrine tumor of the vermiform appendix with uncertain clinical behavior. It was first described by Gagne and Subbuswamy in 1969 and 1974, respectively. The tumor occurs almost exclusively in the vermiform appendix. We present a case of a 60-year-old female, who was referred to the Bielanski Hospital with signs and symptoms of acute appendicitis. Microscopic examination of the appendix showed features of acute appendicitis, however scattered groups of cells with clear cytoplasm as well as strands of single cells with no evidence of atypia were seen. The patient underwent a right  hemicolectomy due to the diagnosis of GCC.

 

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[191]

TÍTULO / TITLE:  - Prognostic significance of AKT/mTOR signaling in advanced neuroendocrine tumors treated with somatostatin analogs.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Onco Targets Ther. 2012;5:409-16. doi: 10.2147/OTT.S36330. Epub 2012 Nov 28.

            ●● Enlace al texto completo (gratuito o de pago) 2147/OTT.S36330

AUTORES / AUTHORS:  - Fernandes I; Pacheco TR; Costa A; Santos AC; Fernandes AR; Santos M; Oliveira AG; Casimiro S; Quintela A; Fernandes A; Ramos M; Costa L

INSTITUCIÓN / INSTITUTION:  - Department of Medical Oncology, Hospital Santa Maria, CHLN, Lisboa, Portugal; ; Clinical and Translational Oncology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisboa, Portugal;

RESUMEN / SUMMARY:  - INTRODUCTION: Somatostatin analogs (SSAs) are used as part of standard treatment  for advanced neuroendocrine tumors (NETs). The mechanisms behind the antiproliferative action of SSAs remain largely unknown, but a connection with the mammalian target of rapamycin (mTOR) signaling pathway has been suggested. Our purpose was to evaluate the activation status of the AKT/mTOR pathway in advanced metastatic NETs and identify biomarkers of response to SSA therapy. PATIENTS AND METHODS: Expression of phosphatase and tensin homolog (PTEN), phosphorylated (p)-AKT(Ser473), and p-S6(Ser240/244) was evaluated using immunohistochemistry in archival paraffin samples from 23 patients. Expression levels were correlated with clinicopathological parameters and progression-free survival under treatment with SSAs. RESULTS: A positive association between p-AKT and p-S6 expression was identified (P = 0.01) and higher expression of both markers was observed in pancreatic NETs. AKT/mTOR activation was observed without the loss of PTEN expression. Tumors showing AKT/mTOR signaling activation progressed faster when treated with SSAs: higher expression of p-AKT or p-S6 predicted a median progression-free survival of 1 month vs 26.5 months for lower  expression (P = 0.02). CONCLUSION: Constitutive activation of the AKT/mTOR pathway was associated with shorter time-to-progression in patients undergoing treatment with SSAs. Larger case series are needed to validate whether p-AKT(Ser473) and p-S6(Ser240/244) can be used as prognostic markers of response  to therapy with SSAs.

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[192]

TÍTULO / TITLE:  - Clinical management and outcomes of papillary, follicular and medullary thyroid cancer surgery.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Med Glas Ljek komore Zenicko-doboj kantona. 2013 Feb;10(1):164-7.

AUTORES / AUTHORS:  - Rahmani N; Abbas Hashemi S; Fazli M; Raisian M

INSTITUCIÓN / INSTITUTION:  - 1Department of General Surgery, 2Student Research Commit- tee; School of Medicine, Mazandaran University of Medical Sciences, Sari, Iran.

RESUMEN / SUMMARY:  - The clinical characteristics, pathological subtypes and patients’ survival in 40  patients with thyroid carcinoma between March 2007 and March 2012 were evaluated. This study included 33 (82.5 %) females and seven (17.5%) males (female to male ratio of 4.7:1). The median age of patients was 47.5 (range; 24-64). Papillary carcinoma was the commonest pathological subtype (23 patients, 57.5%), followed by follicular carcinoma (14 patients, 35%) and medullary carcinoma (3 cases, 7.5%). Total thryoidectomy was performed in 30 (75%), lobectomy in six (15%), subtotal and multifocal thryoidectomy in two (5%) patients. The median time of follow up was 3 years with range of 1-5 years. After ive years 34 (85%) patients  were alive and six (15%) were dead. The overall 5-year actuarial survival was 85%, for papillary carcinoma 91.3%, for follicular carcinoma 85.7% and for medullary carcinoma it was 33.3%. The results suggest that total thryoidectomy had better outcome in comparison with other surgeries.

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[193]

TÍTULO / TITLE:  - Prolonged Survival in an Aged Labrador Retriever with a Metastatic Insulinoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Am Anim Hosp Assoc. 2013 Jan 17.

            ●● Enlace al texto completo (gratuito o de pago) 5326/JAAHA-MS-5860

AUTORES / AUTHORS:  - Rychel J; Worley DR; Hardy CS; Webb BT

INSTITUCIÓN / INSTITUTION:  - Fort Collins Veterinary Emergency and Rehabilitation Hospital, Fort Collins, CO,  Colorado State University, Fort Collins, CO.

RESUMEN / SUMMARY:  - This case report highlights an unusually prolonged, asymptomatic, disease-free interval in an aged male Labrador retriever that underwent partial pancreatectomy for a functionally active pancreatic insulinoma with histologically confirmed hepatic metastasis. The patient developed pancreatitis and nonseptic suppurative  peritonitis 24 hr after surgical resection of the insulinoma and was managed medically until discharge. Three mo after surgery, the dog was diagnosed with exocrine pancreatic insufficiency (EPI) that was effectively managed with parenteral pancreatic enzymes. Due to normal glucose levels 3 mo postsurgically,  liver samples from the initial surgery were resubmitted for immunohistochemistry. Results confirmed insulinoma metastasis with insulin expression. Ten mo postsurgically, the blood glucose was normal and serum insulin levels were slightly above the upper reference limit. The first hypoglycemic episode was documented 23 mo postoperatively, which was effectively managed with prednisone.  The cause for the prolonged disease remission and survival was unknown, but was possibly a result of pancreatitis and peritonitis, partial spontaneous regression of metastatic lesions, or idiopathic. Despite life-threatening postoperative complications, this patient enjoyed a profoundly longer than expected survival. This case highlights the importance of removing the primary tumor (insulinoma) despite the presence of metastatic disease.

 

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[194]

TÍTULO / TITLE:  - Primary cardiac neuroendocrine carcinoma and minimally invasive cardiac surgery.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Asian Cardiovasc Thorac Ann. 2012 Dec;20(6):721-3. doi: 10.1177/0218492312440265.

            ●● Enlace al texto completo (gratuito o de pago) 1177/0218492312440265

AUTORES / AUTHORS:  - Carmona P; Lazaro J; Llagunes J; Canovas S

INSTITUCIÓN / INSTITUTION:  - paulac_g@hotmail.com.

RESUMEN / SUMMARY:  - Diagnosis of cardiac neoplasm has become more feasible with the use of echocardiography, but imaging is sometimes not enough to establish a diagnosis, and a biopsy is required to determine the diagnosis and treatment. Transesophageal echocardiography-directed biopsies via a minithoracotomy revealed an extremely rare primary cardiac tumor, a poorly differentiated neuroendocrine carcinoma, in the left atrium of a 54-year-old man who presented with syncope and hypotension.

 

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[195]

TÍTULO / TITLE:  - Pancreatic neuroendocrine tumors: biology, diagnosis, and treatment.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Chin J Cancer. 2012 Dec 14. doi: 10.5732/cjc.012.10295.

            ●● Enlace al texto completo (gratuito o de pago) 5732/cjc.012.10295

AUTORES / AUTHORS:  - Ro C; Chai W; E Yu V; Yu R

INSTITUCIÓN / INSTITUTION:  - Cedars-Sinai Medical Center, Los Angeles, California 90048, USA run.yu@cshs.org.

RESUMEN / SUMMARY:  - Pancreatic neuroendocrine tumors (PNETs), a group of endocrine tumors arising in  the pancreas, are among the most common neuroendocrine tumors. The genetic causes of familial and sporadic PNETs are understood to some degree while the molecular  pathogenesis of PNETs is still unclear. Most PNETs are indolent but have malignant potential. The biological behavior of an individual PNET is not readily predictable; higher tumor grade, lymph node and liver metastasis, and larger tumor size generally portend less favorable prognosis. Endocrine testing, imaging, and histological evidence are needed for accurate diagnosis of PNETs. An “aggressive” treatment approach with 4 components: surgery, locoregional therapy, systemic therapy, and complication control, has becomes a trend in academic centers throughout the world. The optimal use of the multiple modalities of systemic therapy is still being developed; efficacy, safety, availability, and cost should be considered when treating a specific patient. Clinical presentation, diagnosis, and treatment of specific types of PNETs and familial PNET syndromes, including a novel Mahvash disease, are summarized.

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[196]

TÍTULO / TITLE:  - Endoscopic treatment of bronchial carcinoids in comparison to surgical resection: a retrospective study.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Bronchology Interv Pulmonol. 2012 Jan;19(1):29-34. doi: 10.1097/LBR.0b013e3182446b52.

            ●● Enlace al texto completo (gratuito o de pago) 1097/LBR.0b013e3182446b52

AUTORES / AUTHORS:  - Neyman K; Sundset A; Naalsund A; Espinoza A; Solberg S; Kongerud J; Fosse E

INSTITUCIÓN / INSTITUTION:  - The Intervention Centre, Rikshospitalet, Sognsvannsveien 20, Oslo, Norway. kirill.neyman@gmail.com

RESUMEN / SUMMARY:  - BACKGROUND: Surgery is the gold standard of lung carcinoid treatment. However, bronchoscopic treatment may provide a complete cure in selected patients. The aim of the study was to review the results of laser treatment of bronchial carcinoids and to compare the outcome after laser resection against the outcome after surgical resection. METHODS: Seventy-three patients, 29 men and 44 women, median  age 53 years (range, 23 to 78 y), with bronchial carcinoids were treated by surgical resection (n=48) or endobronchial ablation (n=25). Bronchoscopic treatment was also performed in 5 of 48 surgical patients as a part of the surgical treatment strategy. RESULTS: Among 25 patients treated endoscopically, 16 were successfully treated with laser, whereas 9 were operated subsequently. One major complication was registered, as an inadvertent ventilation caused a nonfatal fire of the bronchoscope during Nd:YAG laser procedure. Forty-eight patients underwent surgical resection. Most of the patients underwent lobectomy and bilobectomy (30 and 5 patients, respectively). Four of the patients were dead by the end of the study, 1 was treated with laser, and 3 treated with surgical resection. The overall survival was 94.5% in the surgical group and 94.4% in the  group treated with endoscopic ablation (P=0.9). None of the 69 survivors had any  sign of recurrence on computed tomographic scans and bronchoscopy by the end of the study. CONCLUSIONS: This is a retrospective study and no randomization has been performed. However, the results add evidence to the view that transbronchial laser treatment may be offered as a safe, stand-alone procedure in the treatment  of typical carcinoid tumor in the central airways.

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[197]

TÍTULO / TITLE:  - Strumal carcinoid tumour of the ovary presenting with severe constipation.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Singapore Med J. 2013 Jan;54(1):e21-3.

AUTORES / AUTHORS:  - Sulaiman S; Chia YN; Namuduri RD

INSTITUCIÓN / INSTITUTION:  - Department of Obstetrics and Gynaecology, KK Women’s and Children’s Hospital, 100 Bukit Timah Road, Singapore 229899. suzanna@doctors.org.uk.

RESUMEN / SUMMARY:  - A 30-year-old single woman presented with an incidental finding of abdominal mass associated with severe constipation. Her cancer antigen-25, alpha-foetoprotein and beta human chorionic gonadotropin levels were normal, but her carcinoembryonic antigen level was raised at 7.6 g/dL. Magnetic resonance imaging showed a 11.4 cm x 8.6 cm x 9.5 cm right ovarian mass with solid and cystic areas. An open right cystectomy was performed. Intraoperatively, she was found to have hirsutism and clitoromegaly. During the operation, there was a right ovarian 10-cm mass, with faecal loading from the caecum to the transverse colon. The uterus, fallopian tubes, left ovary and intraperitoneal survey were normal. Final histology confirmed strumal carcinoid tumour Stage 1A. This case report shows that a strumal carcinoid tumour can present with longstanding constipation as a patient’s main complaint and may also be associated with hirsutism.

 

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[198]

TÍTULO / TITLE:  - Which are the cells of origin in merkel cell carcinoma?

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Skin Cancer. 2012;2012:680410. doi: 10.1155/2012/680410. Epub 2012 Dec 13.

            ●● Enlace al texto completo (gratuito o de pago) 1155/2012/680410

AUTORES / AUTHORS:  - Tilling T; Moll I

INSTITUCIÓN / INSTITUTION:  - Department of Dermatology, University Medical Center Hamburg-Eppendorf, Martinistrasse 52, 20246 Hamburg, Germany.

RESUMEN / SUMMARY:  - Merkel cell carcinoma (MCC), a highly aggressive skin tumour with increasing incidence, is associated with the newly discovered Merkel cell polyomavirus (MCPyV). Studies on MCC and MCPyV as well as other risk factors have significantly increased our knowledge of MCC pathogenesis, but the cells of origin, which could be important targets in future therapies, are still unknown.  Merkel cells (MCs), the neuroendocrine cells of the skin, were believed to be at  the origin of MCC due to their phenotypic similarities. However, for several reasons, for example, heterogeneous differentiation of MCCs and postmitotic character of MCs, it is not very likely that MCC develops from differentiated MCs. Skin stem cells, probably from the epidermal lineage, are more likely to be  cells of origin in MCC. Future studies will have to address these questions more  directly in order to identify the physiological cells which are transformed to MCC cells.

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[199]

TÍTULO / TITLE:  - Surgical treatment of multiple spine metastases from gastrinoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Evid Based Spine Care J. 2011 Nov;2(4):45-50. doi: 10.1055/s-0031-1274756.

            ●● Enlace al texto completo (gratuito o de pago) 1055/s-0031-1274756

AUTORES / AUTHORS:  - Crabtree KL; Anderson KK; Haynes NG; Arnold PM

INSTITUCIÓN / INSTITUTION:  - University of Kansas Medical Center, Kansas City, KS, USA.

RESUMEN / SUMMARY:  - Study design: Case report.Clinical question: To report successful surgical therapy for spinal cord compression in a patient with spinal metastases from a pancreatic gastrinoma.Methods: A 43-year-old man presented three times within 4 years with cervical and upper thoracic spinal cord compression because of metastatic gastrinoma. He had two previous spine metastases to the lower thoracic and lumbar spine, a T11 compressive lesion which required a T9L1 fusion, and an L4 lesion that was treated with chemotherapy and stereotactic radiation. The compression was relieved each time by surgery.Results: The patient underwent three surgeries in 4 years: (1) debulking and removal of the rib head on the left at T3, and debulking of the tumor at T3 with hemilaminectomy and spinal cord decompression with internal fixation from T1-T5 using posterolateral instrumented fusion and allograft; (2) anterior C7 corpectomy with placement of a cage from C7-T1 with both anterior and posterior fusion of C2C7; and (3) T1-T3 laminectomy, T1-T3 exploration of wound, revision of hardware, T1-T3 removal of spinal tumor,  and T3 bilateral transpedicular circumferential decompression. The patient is alive and regained the ability to walk 8 years after initial diagnosis, despite the appearance of spinal metastases 1 year after the diagnosis of liver metastases.Conclusion: Surgery for spinal cord compression in patients with metastatic neuroendocrine tumors can be effective in relieving radicular pain, weakness and numbness, and while not curative can greatly improve quality of life.

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[200]

TÍTULO / TITLE:  - Neuroembolization of metastatic Merkel cell cancer to the face for treatment of Kasabach-Merritt syndrome.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - British Medical J (BMJ). Acceso gratuito al texto completo.

            ●● Enlace a la Editora de la Revista http://bmj.com/search.dtl 

            ●● Cita: British Medical J. (BMJ): <> Case Rep. 2013 Jan 17;2013. pii: bcr2012010556. doi: 10.1136/bcr-2012-010556.

            ●● Enlace al texto completo (gratuito o de pago) 1136/bcr-2012-010556

AUTORES / AUTHORS:  - Lee JH; Roychowdhury S; Nissenblatt MJ

INSTITUCIÓN / INSTITUTION:  - Department of Radiology, UMDNJ Robert Wood Johnson University Hospital, New Brunswick, New Jersey, USA.

RESUMEN / SUMMARY:  - Kasabach-Merritt syndrome is defined as a consumptive thrombocytopenia in the presence of a highly vascular tumor. Multiple treatment options, including transarterial embolization, have been described. We demonstrate that transarterial embolization is a viable option in the treatment of a rapidly progressive and debilitating Merkel cell tumor metastasizing to the head and neck presenting with Kasabach-Merritt syndrome.

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[201]

TÍTULO / TITLE:  - Treatment of neuroendocrine tumor liver metastases.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Int J Hepatol. 2012;2012:973946. doi: 10.1155/2012/973946. Epub 2012 Nov 25.

            ●● Enlace al texto completo (gratuito o de pago) 1155/2012/973946

AUTORES / AUTHORS:  - Lewis MA; Hobday TJ

INSTITUCIÓN / INSTITUTION:  - Division of Medical Oncology, Mayo Clinic, Rochester, MN 55905, USA.

RESUMEN / SUMMARY:  - In the care of patients with hepatic neuroendocrine metastases, medical oncologists should work in multidisciplinary fashion with surgeons, interventional radiologists, and radiation oncologists to assess the potential utility of liver-directed and systemic therapies. This paper addresses the various roles and evidence basis for cytoreductive surgery, thermal ablation (radiofrequency, microwave, and cryoablation), and embolization (bland embolization (HAE), chemoembolization (HACE), and radioembolization) as liver-directed therapies. Somatostatin analogues, cytotoxic chemotherapy, and the newer agents everolimus and suntinib are discussed as a means for controlling intra- and extrahepatic disease, along with peptide receptor radiotherapy (PRRT). Finally, the experience with orthotopic liver transplant for neuroendocrine tumors is described.

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[202]

TÍTULO / TITLE:  - Profile of cabozantinib and its potential in the treatment of advanced medullary  thyroid cancer.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Onco Targets Ther. 2013;6:1-7. doi: 10.2147/OTT.S27671. Epub 2013 Jan 3.

            ●● Enlace al texto completo (gratuito o de pago) 2147/OTT.S27671

AUTORES / AUTHORS:  - Hart CD; De Boer RH

INSTITUCIÓN / INSTITUTION:  - Department of Oncology, Royal Melbourne Hospital, Victoria, Australia.

RESUMEN / SUMMARY:  - Medullary thyroid cancer is an uncommon malignancy for which until recently little effective treatment existed. It is often characterized by mutation and overexpression of the receptor tyrosine kinases RET (rearranged during transfection), VEGFR2 (vascular endothelial growth factor receptor 2) and MET (mesenchymal-epithelial transition factor), which make attractive targets for drug development. Cabozantinib is an orally bioavailable tyrosine kinase inhibitor which blocks MET, VEGRF2 and RET, and has shown considerable activity in medullary thyroid cancer in a Phase III trial, including in heavily pretreated patients. Its novel combination of vascular endothelial growth factor and MET inhibition is believed to address the MET escape pathway, which is thought to be  the cause of nonsustained tumor responses resulting from inhibition of vascular endothelial growth factor alone.

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[203]

TÍTULO / TITLE:  - Clinical impact of endoscopic submucosal dissection for gastric neuroendocrine tumors: a retrospective study from mainland of china.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - ScientificWorldJournal. 2012;2012:869769. doi: 10.1100/2012/869769. Epub 2012 Dec 23.

            ●● Enlace al texto completo (gratuito o de pago) 1100/2012/869769

AUTORES / AUTHORS:  - Chen WF; Zhou PH; Li QL; Xu MD; Yao LQ

INSTITUCIÓN / INSTITUTION:  - Endoscopy Center and Endoscopy Research Institute, Zhongshan Hospital, Fudan University, 180 FengLin Road, Shanghai, Xuhui 200032, China.

RESUMEN / SUMMARY:  - As a minimally invasive technique, endoscopic resection may benefit patients diagnosed with early stage gastrointestinal neuroendocrine tumors (NETs). However, no studies have yet been published in which endoscopic submucosal dissection (ESD) has been applied for gastric NETs. For the first time a research group in China applied ESD to remove gastric NETs, and indicated that ESD should  be considered for treatment of eligible gastric NETs because the technique shows  a high histologically complete resection rate, provides accurate histopathological evaluation, has a low complication rate, and can be performed within a reasonable timeframe.

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[204]

TÍTULO / TITLE:  - Neuroendocrine tumours in 2012: Insights into signalling pathways could individualize therapy.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Nat Rev Endocrinol. 2013 Feb;9(2):70-2. doi: 10.1038/nrendo.2012.250. Epub 2013 Jan 8.

            ●● Enlace al texto completo (gratuito o de pago) 1038/nrendo.2012.250

AUTORES / AUTHORS:  - Oberg K

INSTITUCIÓN / INSTITUTION:  - Department of Endocrine Oncology, University Hospital of Uppsala, Entrance 78D, 1st floor, 75185 Uppsala, Sweden. kjell.oberg@medsci.uu.se.

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[205]

TÍTULO / TITLE:  - Neuroendocrine carcinoma of the stomach: clinicopathological and immunohistochemical evaluation.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Med Mol Morphol. 2013 Jan 10.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s00795-012-0006-8

AUTORES / AUTHORS:  - Namikawa T; Oki T; Kitagawa H; Okabayashi T; Kobayashi M; Hanazaki K

INSTITUCIÓN / INSTITUTION:  - Department of Surgery, Kochi Medical School, Kohasu, Oko-cho, Nankoku, Kochi, 783-8505, Japan, tsutomun@kochi-u.ac.jp.

RESUMEN / SUMMARY:  - Neuroendocrine carcinoma (NEC) of the stomach is an uncommon disease. Because of  its rarity, the clinicopathological features are unclear, and there is no consensus on the optimal treatment strategy. This study included five consecutive patients with gastric NEC who underwent surgery from July 2001 to August 2011. Clinical presentation, tumor location, tumor morphology and size, pathology and immunohistochemistry results, and treatment outcome were analyzed retrospectively and discussed. The study cohort of four men and one woman ranged in age from 52 to 84 years, with a median age of 72 years. Positive rates of neuroendocrine markers were 40 % for chromogranin A, 60 % for synaptophysin, 60 % for CD56, 40 % for neuron-specific enolase, and 100 % for p53 protein. Median number of lymph node metastases per patient was 10, with severe lymphatic and venous infiltration, and high Ki-67 labeling index (60-90 %) reported for all patients.  Median tumor size was 6 cm. Stage IV disease was diagnosed in three patients; the other two patients showed stage IIIA tumors. After a mean follow-up of 29.8 months, two of the five patients had died of the disease. Although rare, gastric  NECs deserve particular attention because of their strong malignant potential associated with an extremely poor prognosis. Such carcinomas demand an aggressive surgical approach followed by chemotherapy and multimodality adjuvant therapy.

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[206]

TÍTULO / TITLE:  - A rare case of adult hepatoblastoma with neuroendocrine differentiation misdiagnosed as neuroendocrine tumor.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Int J Clin Exp Pathol. 2013;6(2):308-13. Epub 2013 Jan 15.

AUTORES / AUTHORS:  - Zhang Q; Ming J; Zhang S; Guo D; Qiu X

INSTITUCIÓN / INSTITUTION:  - Department of Pathology, the First Affiliated Hospital and College of Basic Medical Sciences, China Medical University Shenyang, PR China.

RESUMEN / SUMMARY:  - Hepatoblastoma is the most common malignant liver tumor between the age of 6 months and 3 years, but it is extremely rare in adults. Only Forty-five cases of  adult hepatoblastoma were reported up to now in the world. Hepatoblastoma exhibits a wide range of epithelial and mesenchymal lines differentiation, but neuroendocrine differentiation of this tumor has rare been reported in the literature. Here, we reported a case of primary giant hepatoblastoma (about 11.4*7.9*15.3cm) with neuroendocrine differentiation of a 32-year-old woman, which was misdiagnosed as neuroendocrine tumor.

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[207]

TÍTULO / TITLE:  - Immunohistochemical expression of PAX5 and TdT by Merkel cell carcinoma and pulmonary small cell carcinoma: a potential diagnostic pitfall but useful discriminatory marker.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Int J Clin Exp Pathol. 2013;6(2):142-7. Epub 2013 Jan 15.

AUTORES / AUTHORS:  - Kolhe R; Reid MD; Lee JR; Cohen C; Ramalingam P

INSTITUCIÓN / INSTITUTION:  - Department of Pathology, Georgia Health Sciences University Augusta, GA.

RESUMEN / SUMMARY:  - BACKGROUND: Merkel cell carcinoma is a high-grade neuroendocrine carcinoma of skin that is characterized by immature cells which, because of its striking morphologic similarity, may be confused with other small round blue cell tumors such as pulmonary small cell carcinoma or lymphoblastic leukemia/lymphoma. Immunohistochemistry is therefore paramount to ensuring accurate diagnostic distinction between these tumors. The aim of our study was to evaluate and compare the expression of PAX5 and Terminal deoxynucleotidyl transferase (TdT), in Merkel cell carcinoma and pulmonary small cell carcinoma. DESIGN: PAX5 and TdT immunohistochemical stains were performed on 27 Merkel cell carcinomas and 10 pulmonary small cell carcinomas. RESULTS: PAX5 was expressed in 24/27 (89%) Merkel cell carcinomas and 0/10 (0%) pulmonary small cell carcinomas. TdT was expressed in 21/27 (78%) Merkel cell carcinomas and 9/10 (90%) pulmonary small cell carcinomas. CONCLUSIONS: Our study confirms that PAX5 and TdT expression can be expressed in a high percentage of Merkel cell carcinomas and so when positive  are not diagnostic of lymphoblastic leukemia/lymphoma. When dealing with metastatic lesions, PAX5 negativity would favor a diagnosis of pulmonary small cell carcinoma over Merkel cell carcinoma. In addition, TTF-1 negative pulmonary  small cell carcinoma is to be differentiated from Merkel cell carcinoma.

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[208]

TÍTULO / TITLE:  - Neurofibromatosis Type 2 Tumor Suppressor Protein, NF2, Induces Proteasome-Mediated Degradation of JC Virus T-Antigen in Human Glioblastoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - PLoS One. 2013;8(1):e53447. doi: 10.1371/journal.pone.0053447. Epub 2013 Jan 7.

            ●● Enlace al texto completo (gratuito o de pago) 1371/journal.pone.0053447

AUTORES / AUTHORS:  - Beltrami S; Branchetti E; Sariyer IK; Otte J; Weaver M; Gordon J

INSTITUCIÓN / INSTITUTION:  - Department of Neuroscience and Center for Neurovirology, Temple University School of Medicine, Philadelphia, Pennsylvania, United States of America ; Biomedical Neuroscience Graduate Program, Temple University School of Medicine, Philadelphia, Pennsylvania, United States of America.

RESUMEN / SUMMARY:  - Neurofibromatosis type 2 protein (NF2) has been shown to act as tumor suppressor  primarily through its functions as a cytoskeletal scaffold. However, NF2 can also be found in the nucleus, where its role is less clear. Previously, our group has  identified JC virus (JCV) tumor antigen (T-antigen) as a nuclear binding partner  for NF2 in tumors derived from JCV T-antigen transgenic mice. The association of  NF2 with T-antigen in neuronal origin tumors suggests a potential role for NF2 in regulating the expression of the JCV T-antigen. Here, we report that NF2 suppresses T-antigen protein expression in U-87 MG human glioblastoma cells, which subsequently reduces T-antigen-mediated regulation of the JCV promoter. When T-antigen mRNA was quantified, it was determined that increasing expression  of NF2 correlated with an accumulation of T-antigen mRNA; however, a decrease in  T-antigen at the protein level was observed. NF2 was found to promote degradation of ubiquitin bound T-antigen protein via a proteasome dependent pathway concomitant with the accumulation of the JCV early mRNA encoding T-antigen. The interaction between T-antigen and NF2 maps to the FERM domain of NF2, which has been shown previously to be responsible for its tumor suppressor activity. Co-immunoprecipitation assays revealed a ternary complex among NF2, T-antigen, and the tumor suppressor protein, p53 within a glioblastoma cell line. Further, these proteins were detected in various degrees in patient tumor tissue, suggesting that these associations may occur in vivo. Collectively, these results demonstrate that NF2 negatively regulates JCV T-antigen expression by proteasome-mediated degradation, and suggest a novel role for NF2 as a suppressor of JCV T-antigen-induced cell cycle regulation.

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[209]

TÍTULO / TITLE:  - Confocal scan imaging and impression cytology of the cornea in a case of multiple endocrine neoplasia type-2b.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Ophthalmic Vis Res. 2012 Apr;7(2):176-9.

AUTORES / AUTHORS:  - Javadi MA; Rezaei Kanavi M; Faramarzi A; Feizi S; Azizi F; Javadi F

INSTITUCIÓN / INSTITUTION:  - Ophthalmic Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

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[210]

TÍTULO / TITLE:  - Plasma-free metanephrine and free normetanephrine measurement for the diagnosis of pheochromocytoma in dogs.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Vet Intern Med. 2013 Jan;27(1):83-90. doi: 10.1111/jvim.12009.

            ●● Enlace al texto completo (gratuito o de pago) 1111/jvim.12009

AUTORES / AUTHORS:  - Gostelow R; Bridger N; Syme HM

INSTITUCIÓN / INSTITUTION:  - Department of Veterinary Clinical Sciences, The Royal Veterinary College, Hertfordshire, UK.

RESUMEN / SUMMARY:  - BACKGROUND: Measurement of plasma-free metanephrines is the test of choice to identify pheochromocytoma in human patients. OBJECTIVES: To establish the sensitivity and specificity of plasma-free metanephrine (fMN) and free normetanephrine (fNMN) concentrations to diagnose pheochromocytoma in dogs. ANIMALS: Forty-five client-owned dogs (8 dogs with pheochromocytoma, 11 dogs with adrenocortical tumors, 15 dogs with nonadrenal disease, and 11 healthy dogs.) METHODS: A prospective study. EDTA plasma was collected from diseased and healthy dogs and submitted for fMN and fNMN measurement by liquid chromatography-tandem mass spectrometry (LC-MS/MS). RESULTS: Free MN concentration (median [range]) was significantly higher in dogs with pheochromocytoma (8.15 [1.73-175.23] nmol/L) than in healthy dogs (0.95 [0.68-3.08] nmol/L; P < .01) and dogs with adrenocortical tumors (0.92 [0.25-2.51] nmol/L; P < .001), but was not different  from dogs with nonadrenal disease (1.91 [0.41-6.57] nmol/L; P >/= .05). Free NMN  concentration was significantly higher in dogs with pheochromocytoma (63.89 [10.19-190.31] nmol/L) than in healthy dogs (2.54 [1.59-4.17] nmol/L; P < .001),  dogs with nonadrenal disease (3.30 [1.30-10.10] nmol/L; P < .001), and dogs with  adrenocortical tumors (2.96 [1.92-5.01] nmol/L); P < 0.01). When used to diagnose pheochromocytoma, a fMN concentration of 4.18 nmol/L had a sensitivity of 62.5% and specificity of 97.3%, and a fNMN concentration of 5.52 nmol/L had a sensitivity of 100% and specificity of 97.6%. CONCLUSIONS AND CLINICAL IMPORTANCE: Plasma fNMN concentration has excellent sensitivity and specificity for the diagnosis of pheochromocytoma in dogs, whereas fMN concentration has moderate sensitivity and excellent specificity. Measurement of plasma-free metanephrines provides an effective, noninvasive, means of identifying dogs with  pheochromocytoma.

 

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[211]

TÍTULO / TITLE:  - Intraoperative diagnosis of functional retroperitoneal multiple paraganglioma: A  case report.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Oncol Lett. 2012 Oct;4(4):829-831. Epub 2012 Jul 10.

            ●● Enlace al texto completo (gratuito o de pago) 3892/ol.2012.795

AUTORES / AUTHORS:  - Guo Q; Li B; Guan J; Yang H; Wu Y

INSTITUCIÓN / INSTITUTION:  - Department of Surgery, The Second Affiliated Hospital, College of Medicine, Zhejiang University, Zhejiang 310009;

RESUMEN / SUMMARY:  - Paragangliomas are extra-adrenal chromaffin tumors that arise from neuroectodermal cells of the autonomous nervous system. It is difficult to make an accurate preoperative clinical diagnosis of silent paraganglioma. The best choice of treatment is complete surgical resection. However, it is important to note that in patients with functional paragangliomas, the tumor’s ability to produce catecholamines may cause abrupt changes in blood pressure. Thus, surgery  may induce life-threatening complications. In the present study, we present a case of functional retroperitoneal multiple paraganglioma in a 39-year-old male patient who was diagnosed during surgery. Four years after the operation, the patient remains asymptomatic and free of disease.

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[212]

TÍTULO / TITLE:  - Correlation of monoclonal and polyclonal somatostatin receptor 5 antibodies in pancreatic neuroendocrine tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Int J Clin Exp Pathol. 2013;6(1):49-54. Epub 2012 Nov 20.

AUTORES / AUTHORS:  - Kaemmerer D; Lupp A; Peter L; Fischer E; Schulz S; Kloppel G; Hommann M

INSTITUCIÓN / INSTITUTION:  - Department of General and Visceral Surgery, Zentralklinik Bad Berka Bad Berka, Germany. Daniel.Kaemmerer@zentralklinik.de

RESUMEN / SUMMARY:  - AIMS: To evaluate the frequency of somatostatin-receptor 5 (SSTR 5) in pancreatic neuroendocrine tumors by using monoclonal and polyclonal antibodies. MATERIAL AND METHOD: we analyzed 66 proven pancreatic neuroendocrine tumors immunohistochemically with monoclonal (clone UMB-4) and polyclonal SSTR 5-antibodies. Immunoreactive score (IRS) and DAKO-score Her2/neu were evaluated.  RESULTS: Immunohistochemistry analysis demonstrated for the IRS a significant higher staining of all specimen using the monoclonal antibodies ( IRS SSTR5 poly  vs IRS SSTR 5 mono; 20.0% vs 30.3% p < 0.001) by a correlation of 0.21; p = 0.04. For the HER2 score there was also a significant higher staining in the monoclonal group (Her2 SSTR 5 poly vs Her2 SSTR 5 mono; 21.5% vs 28.8% p < 0.001) by a correlation of 0.20; p = 0.08. CONCLUSION: Both antibodies are useful in staining of SSTR, although UMB-4 demonstrated a 10% higher SSTR 5 staining. Due to the previous underestimated expression rate of SSTR 5, current standards in diagnostics and therapy should be reconsidered. The increasing usage of long-acting pansomatostatin receptor analogues will rise the adverse effects connected to SSTR5 binding.

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[213]

TÍTULO / TITLE:  - Laparoscopic adrenalectomy for pheochromocytoma in a child.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - APSP J Case Rep. 2013 Jan;4(1):2. Epub 2013 Jan 1.

AUTORES / AUTHORS:  - Soheilipour F; Pazouki A; Ghorbanpour S; Tamannaie Z

INSTITUCIÓN / INSTITUTION:  - Assistant Professor of Pediatric Endocrinology and Metabolism, Minimally Invasive Surgery Research Center, Rasoul Akram Hospital, Tehran University of Medical Sciences, Tehran, Iran.

RESUMEN / SUMMARY:  - Pheochromocytoma is a catecholamine-secreting tumor of the adrenal medulla. It has wide and subtle range of clinical manifestations including sustained hypertension in about 1% of pediatric patients. Although laparoscopic adrenalectomy is the gold standard treatment method in adult patients, few reports have described this technique in children. We report a child with unilateral pheochromocytoma who presented with poor weight gain, polyuria and polydipsia. Diagnosis was based upon clinical and laboratory evaluation. She was  treated successfully by laparoscopic adrenalectomy.

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[214]

TÍTULO / TITLE:  - A case report of a malignant peripheral nerve sheath tumor of the oral cavity in  neurofibromatosis type 1.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Case Report Otolaryngol. 2012;2012:936735. doi: 10.1155/2012/936735. Epub 2012 Nov 6.

            ●● Enlace al texto completo (gratuito o de pago) 1155/2012/936735

AUTORES / AUTHORS:  - Ozturk O; Tutkun A

INSTITUCIÓN / INSTITUTION:  - Department of Otorhinolaryngology, School of Medicine, Istanbul Medipol University, Istanbul, Turkey ; Kulak Burun Bogaz Anabilim Dali, Istanbul Medipol  Universitesi, Kosuyolu, Kadikoy, 34718 Istanbul, Turkey.

RESUMEN / SUMMARY:  - Patients with neurofibromatosis type 1 develop both benign and malignant tumors at an increased frequency. Most of the malignant peripheral nerve sheath tumors (MPNSTs) are considered as high-grade sarcomas originating from tissues of mesenchymal origin. It is generally accepted that MPNSTs occur in about 2% to 5%  of neurofibromatosis patients. In this paper, we present a 16-year-old male patient with neurofibromatosis who developed MPNST of the retromolar area. The mass enlarged rapidly in a period of 6 weeks. The patient was treated surgically, and a tumor mass with a diameter of 7 x 6 x 4 cm was excised, but after 8 months  a recurrence was observed at the same site. The sarcomatous change in a neurofibroma has an extremely poor prognosis, so patients with neurofibromatosis  should be closely monitored for a possible malignancy. A rapid change in size of  a preexisting neurofibroma, infiltration of the adjacent structures, intralesional hemorrhage, and pain indicate a possible malignant transformation to MPNST.

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[215]

TÍTULO / TITLE:  - Age-specific seroprevalence of Merkel cell polyomavirus, Human polyomaviruses 6,  7 and 9 and Trichodysplasia Spinulosa-associated polyomavirus.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Clin Vaccine Immunol. 2013 Jan 9.

            ●● Enlace al texto completo (gratuito o de pago) 1128/CVI.00438-12

AUTORES / AUTHORS:  - Nicol JT; Robinot R; Carpentier A; Carandina G; Mazzoni E; Tognon M; Touze A; Coursaget P

INSTITUCIÓN / INSTITUTION:  - Universite Francois Rabelais, Laboratory of Molecular Virology and Immunology, Tours, France.

RESUMEN / SUMMARY:  - Six new human polyomaviruses have been identified since 2008 (MCPyV, HPyV6, HPyV7, HPyV9, TSPyV and MWPyV). The presence of specific antibodies against MCPyV, HPyV6, HPyV7, HPyV9 and TSPyV was investigated by virus-like particle based ELISAs in 828 Italian subjects aged 1 to 100 years. The findings indicated  that all these new polyomaviruses circulate widely in humans, with seroprevalence in adulthood ranging from 39.4% for HPyV9 to 87.1% for MCPyV, and that primary exposure is most intense in childhood, with the exception of HPyV7 and HPyV9 for  which the seroprevalence increased throughout life. The proportion of subjects with high antibody titers was found to increase with age for MCPyV and decrease with age for TSPyV.

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[216]

TÍTULO / TITLE:  - Chromophobe renal cell carcinoma, oncocytic variant: a proposal of a new variant  giving a critical diagnostic pitfall in diagnosing renal oncocytic tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Med Mol Morphol. 2013 Jan 22.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s00795-012-0007-7

AUTORES / AUTHORS:  - Kuroda N; Tanaka A; Yamaguchi T; Kasahara K; Naruse K; Yamada Y; Hatanaka K; Shinohara N; Nagashima Y; Mikami S; Oya M; Hamashima T; Michal M; Hes O

INSTITUCIÓN / INSTITUTION:  - Department of Diagnostic Pathology, Kochi Red Cross Hospital, Shin-honmachi 2-13-51, Kochi, Kochi, 780-8562, Japan, kurochankochi@yahoo.co.jp.

RESUMEN / SUMMARY:  - In chromophobe renal cell carcinoma (RCC), two forms of typical and eosinophilic  variants have been reported to date. We have previously reported a new variant of chromophobe RCC, namely an oncocytic variant. However, little is known on the histological features of this variant. In this article, we report such five cases. Macroscopically, the tumor was well demarcated, but unencapsulated. The cut surface of the tumor showed brown in color, but neither hemorrhage nor necrosis was seen. Microscopically, the tumor consisted of predominant tubular configuration with or without various proportion of solid-sheet pattern. In one tumor, tumor cells microscopically invaded branches of renal vein. In addition, the constituting cells were characterized by the oncocytic cytoplasm, trivial to  minimal variation in tumor size, indistinct to slightly distinct cell border, centrally located round nuclei and the absence of perinuclear halo. These characteristics entirely resembled renal oncocytoma. However, neoplastic cells immunohistochemically showed the diffuse and strong labeling for cytokeratin 7 and mitochondrial antigen in all cases. In addition, in fluorescence in situ hybridization (FISH) study the loss of more than four chromosomes among chromosomes 7, 10, 13, 17 and 21 was confirmed in all tumors and the diagnosis of chromophobe RCC was rendered. In conclusion, we propose a new variant, namely an  oncocytic variant, of chromophobe RCC morphologically resembling renal oncocytoma and biologically showing characteristics of chromophobe RCC, and this recognition is practically crucial in the differential diagnosis from renal oncocytoma.

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[217]

TÍTULO / TITLE:  - Functioning unilateral adrenocortical carcinoma in a dog.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Can Vet J. 2012 Jun;53(6):623-5.

AUTORES / AUTHORS:  - Gojska-Zygner O; Lechowski R; Zygner W

INSTITUCIÓN / INSTITUTION:  - Centre of Small Animal Health, Clinic Multiwet, Warsaw, Poland. olgazygner@yahoo.pl

RESUMEN / SUMMARY:  - An 11-year-old, 24-kg, intact female Siberian husky dog in anestrus had a 2-month history of polyuria and polydipsia. The dog had signs of mineralocorticoid excess such as hypertension and hypokalemia refractory to potassium supplementation. Abdominal ultrasound revealed an irregular mass in the left adrenal gland. The ACTH stimulation test for aldosterone concentration did not reveal hyperaldosteronism. Unilateral adrenalectomy was performed and histopathology identified adrenal cortical carcinoma. All clinical signs of mineralocorticoid excess ceased after surgery.

 

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[218]

TÍTULO / TITLE:  - Dexmedetomidine and remifentanil in the perioperative management of an adolescent undergoing resection of pheochromocytoma -A case report-.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Korean J Anesthesiol. 2012 Dec;63(6):555-8. doi: 10.4097/kjae.2012.63.6.555. Epub 2012 Dec 14.

            ●● Enlace al texto completo (gratuito o de pago) 4097/kjae.2012.63.6.555

AUTORES / AUTHORS:  - Jung JW; Park JK; Jeon SY; Kim YH; Nam SH; Choi YG; Bang SR

INSTITUCIÓN / INSTITUTION:  - Department of Anesthesiology and Pain Medicine, Haeundae Paik Hospital, College of Medicine, Inje University, Busan, Korea.

RESUMEN / SUMMARY:  - A 15-year-old adolescent with unilateral multiple adrenal pheochromocytoma had an episode of subcortical intracerebral hemorrhage and seizure 6 weeks before the surgery. He was pretreated with terazosin, losartan, atenolol and levetiracetam for 2 weeks. Dexmedetomidine was started in the preoperative waiting area, and a  combination of dexmedetomidine and remifentanil was continuously infused for most of anesthetic time. To control blood pressure, bolus injection of remifentanil and low-dose infusion of sodium nitroprusside, nicardipine, and esmolol were administered during three adrenergic crises. There was minimal post-resection hypotension, and his trachea was extubated safely 20 min after the surgery. He was discharged without noticeable complication. His catecholamine levels showed the steadily decreasing pattern during the operation in this case. Though a combination of dexmedetomidine and remifentanil may not prevent the hemodynamic instability impeccably during the tumor manipulation, this combination seems to be the way of interrupting release of catecholamines and minimizing hemodynamic fluctuations.

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[219]

TÍTULO / TITLE:  - Tumor-infiltrating lymphocytes and outcome in Merkel cell carcinoma, a virus-associated cancer.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Oncoimmunology. 2012 Nov 1;1(8):1420-1421.

            ●● Enlace al texto completo (gratuito o de pago) 4161/onci.21120

AUTORES / AUTHORS:  - Sihto H; Joensuu H

INSTITUCIÓN / INSTITUTION:  - Laboratory of Molecular Oncology and Molecular Cancer Biology Program; University of Helsinki; Helsinki, Finland.

RESUMEN / SUMMARY:  - An intense immune infiltrate, enriched in T cells, is associated with the presence of Merkel cell polyomavirus (MCPyV) DNA in Merkel cell carcinoma (MCC),  a rare skin cancer. High tumor-infiltrating T-cell counts are associated with favorable survival regardless of the tumor MCPyV status. Hence, boosting host immune functions might constitute a new approach for the treatment of MCC.

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[220]

TÍTULO / TITLE:  - Altered PTEN, ATRX, CHGA, CHGB, and TP53 Expression Are Associated with Aggressive VHL-Associated Pancreatic Neuroendocrine Tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Horm Cancer. 2013 Jan 30.

            ●● Enlace al texto completo (gratuito o de pago) 1007/s12672-013-0134-1

AUTORES / AUTHORS:  - Weisbrod AB; Zhang L; Jain M; Barak S; Quezado MM; Kebebew E

INSTITUCIÓN / INSTITUTION:  - Endocrine Oncology Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD, 20892, USA.

RESUMEN / SUMMARY:  - Von Hippel-Lindau (VHL) syndrome is an inherited cancer syndrome in which 8-17 %  of germline mutation carriers develop pancreatic neuroendocrine tumors (PNETs). There is limited data on prognostic markers for PNETs other than Ki-67, which is  included in the World Health Organization classification system. Recently, specific genes and pathways have been identified by whole exome sequencing which  may be involved in the tumorigenesis of PNETs and may be markers of disease aggressiveness. The objective of this study was to identify molecular markers of  aggressive disease in VHL-associated PNETs. The protein expression of eight genes (PTEN, CHGA, CHGB, ATRX, DAXX, CC-3, VEGF, and TP53) was analyzed in PNETs by immunohistochemistry and compared to clinical data, VHL genotype, functional imaging results, and pathologic findings. Subcellular distribution of phosphatase and tensin (PTEN), chromogranin A (CHGA), and alpha thalassemia/mental retardation syndrome X-linked (ATRX) were significantly different by WHO classifications (p </= 0.05). There was decreased PTEN nuclear to cytoplasmic ratio (p < 0.01) and decreased CHGA nuclear expression (p = 0.03) in malignant samples as compared to benign. Lower cytoplasmic chromogranin B (CHGB) expression (p = 0.03) was associated with malignant tumors and metastasis. Higher nuclear expression of PTEN was associated with VHL mutations in exon 3 (p = 0.04). Higher PTEN and CHGB expression was associated with higher FDG-PET avidity (p < 0.05). Cytoplasmic expression of CC-3 was associated with higher serum chromogranin A levels (rho = 0.72, p = 0.02). Lastly, greater cytoplasmic expression of p53 was  associated with metastasis. Our findings suggest that altered PTEN, ATRX, CHGA, and CHGB expression are associated with aggressive PNET phenotype in VHL and may  serve as useful adjunct prognostic markers to Ki-67 in PNETs.

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[221]

TÍTULO / TITLE:  - Distinct Cytoplasmic Expression of KL-6 Mucin in Chromophobe Renal Cell Carcinoma: A Comparative Immunohistochemical Study with Other Renal Epithelial Cell Tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Acta Histochem Cytochem. 2012 Oct 31;45(5):301-8. doi: 10.1267/ahc.12020. Epub 2012 Sep 11.

            ●● Enlace al texto completo (gratuito o de pago) 1267/ahc.12020

AUTORES / AUTHORS:  - Fukushima M; Higuchi K; Shimojo H; Uehara T; Ota H

INSTITUCIÓN / INSTITUTION:  - Department of Laboratory Medicine, Shinshu University Graduate School of Medicine, 3-1-1 Asahi, Matsumoto, Nagano 390-8621, Japan ; Present address: Department of Pathology and Laboratory Medicine, Kanazawa Medical University, 1-1 Daigaku, Uchinada, Kahoku, Ishikawa 920-0293, Japan.

RESUMEN / SUMMARY:  - The presence of cytoplasmic sialyl glycoproteins is a conspicuous feature in chromophobe renal cell carcinoma (RCC). We compared the immunohistochemical expression of sialyl glycoproteins in chromophobe RCC with that in other types of renal tumors. Formalin-fixed, paraffin-embedded tissues of surgically resected renal tumors (chromophobe RCC, 14 cases [10 cases of classic type and 4 cases of  eosinophilic variant]; oncocytoma, 7 cases; and clear cell RCC, 9 cases) and kidneys from immature infants (4 cases) were immunostained with antibodies against sialyl glycoproteins (anti-KL-6 and anti-sialyl MUC1 antibodies). Cytoplasmic expression of KL-6 and sialyl MUC1 was distinctive in the chromophobe RCC and renal oncocytoma cells, and in the intercalated cells in collecting duct  epithelia. Apical-surface staining of these sialyl glycoproteins was predominantly observed in clear RCC, in the epithelia of the distal tubule and collecting duct, and in the neonatal renal proximal tubule, but not in those of the adult renal proximal tubule. The above-mentioned observations provide additional evidence for similar phenotypic profiles of chromophobe RCC and renal  oncocytoma, and the intercalated cells in collecting ducts and the oncofetal expression of sialyl glycoproteins in clear cell RCC. KL-6 is a potential tumor marker for renal tumors.

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[222]

TÍTULO / TITLE:  - Primary lung carcinoid, a rare cause of paraparesis: report of a case and review  of the literature.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Thorac Dis. 2012 Nov;4(Suppl 1):49-55. doi: 10.3978/j.issn.2072-1439.2012.s005.

            ●● Enlace al texto completo (gratuito o de pago) 3978/j.issn.2072-1439.2012.s005

AUTORES / AUTHORS:  - Visouli AN; Darwiche K; Kourtoglou GI; Zarogoulidis P; Mpakas A; Machairiotis N; Stylianaki A; Christofis C; Katsikogiannis N; Tsakiridis K; Courcoutsakis N; Zarogoulidis K

INSTITUCIÓN / INSTITUTION:  - Cardiothoracic Department, St Luke’s Hospital, Panorama, Thessaloniki, Greece;

RESUMEN / SUMMARY:  - Carcinoids are neuroendocrine tumors mainly involving the gastrointestinal tract, lungs and bronchi. They were considered benign with slow growth, but they can be  malignant in a substantial percentage of patients (metastasizing to liver, bones, skin, etc). Endocrine activity results in carcinoid syndrome. Proximal myopathy has been reported in 7% of patients with carcinoid syndrome. Bronchopulmonary and thymic carcinoids producing adrenocorticotropic hormone can cause Cushing’s syndrome, a main feature of which is myopathy. There are a few reports of carcinoids associated with paraneoplastic neurological syndromes, including neuropathy. We hereby present an extremely rare case of a primary lung carcinoid  presented with paraparesis due to polyneuropathy, and review the relevant literature. To the best of our knowledge there is no similar previous report. Complete resolution of paraparesis after excision of the lung carcinoid suggests  paraneoplastic neurological syndrome.

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[223]

TÍTULO / TITLE:  - Primary neuroendocrine tumor of the left hepatic duct: a case report with review  of the literature.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Case Rep Surg. 2012;2012:786432. doi: 10.1155/2012/786432. Epub 2012 Nov 18.

            ●● Enlace al texto completo (gratuito o de pago) 1155/2012/786432

AUTORES / AUTHORS:  - Bhandarwar AH; Shaikh TA; Borisa AD; Palep JH; Patil AS; Manke AA

INSTITUCIÓN / INSTITUTION:  - Division of GI and HPP Surgery, Department of Surgery, Grant Medical College & Sir JJ Group of Hospitals, Byculla, Mumbai 400008, India.

RESUMEN / SUMMARY:  - Primary Biliary Tract Neuroendocrine tumors (NET) are extremely rare tumors with  only 77 cases been reported in the literature till now. We describe a case of a left hepatic duct NET and review the literature for this rare malignancy. To the  best of our knowledge the present case is the first reported case of a left hepatic duct NET in the literature. In spite of availability of advanced diagnostic tools like Computerized Tomography (CT) Scan and Endoscopic Retrograde Cholangio Pancreaticography (ERCP) a definitive diagnosis of these tumors is possible only after an accurate histopathologic diagnosis of operative specimens  with immunohistochemistry and electron microscopy. Though surgical excision remains the gold standard treatment for such tumors, patients with unresectable tumors have good survival with newer biologic agents like Octreotride.

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[224]

TÍTULO / TITLE:  - A case of early-stage ovarian carcinoid tumor metastasized to the liver.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Case Rep Obstet Gynecol. 2012;2012:961087. doi: 10.1155/2012/961087. Epub 2012 Dec 26.

            ●● Enlace al texto completo (gratuito o de pago) 1155/2012/961087

AUTORES / AUTHORS:  - Kanayama S; Yamada Y; Tanase Y; Haruta S; Nagai A; Kawaguchi R; Yoshida S; Furukawa N; Oi H; Kobayashi H

INSTITUCIÓN / INSTITUTION:  - Department of Obstetrics and Gynecology, Nara Medical University, 840 Shijo-cho,  Nara, Kashihara 634-8522, Japan.

RESUMEN / SUMMARY:  - We report a case of ovarian carcinoid tumor that recurred with multiple liver metastases and was successfully treated with chemoembolization. A 76-year-old woman was admitted to our hospital presented with abdominal distension and abnormal uterine bleeding for about 6 months. She presented with hyperestrogenic  and androgenic manifestations such as vaginal bleeding with endometrial hyperplasia and hirsutism. Magnetic resonance (MR) imaging revealed a large solid and cystic ovarian tumor of 17 cm at maximum diameter. On the basis of the clinical diagnosis of sex cord stromal tumor containing a mature cystic teratoma, she underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy. The pathology report revealed that the mass in the left ovary was a carcinoid tumor, insular type, with mature cystic teratoma. Two years after surgical treatment, multiple liver metastases were revealed by abdominal CT. Hepatic arterial infusion of cisplatin was performed for 2 courses, and multiple metastatic nodules have remarkably reduced. No established chemotherapy or radiation therapy treatments are currently available for recurrent or advanced carcinoid tumors. Our paper suggests that chemoembolization with cisplatin may be effective in treatment of patients with multiple liver metastases of ovarian carcinoid tumor.

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[225]

TÍTULO / TITLE:  - Neoadjuvant chemotherapy with capecitabine and temozolomide for unresectable pancreatic neuroendocrine tumor.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Case Rep Oncol. 2012 Sep;5(3):622-6. doi: 10.1159/000345369. Epub 2012 Sep 20.

            ●● Enlace al texto completo (gratuito o de pago) 1159/000345369

AUTORES / AUTHORS:  - Devata S; Kim EJ

INSTITUCIÓN / INSTITUTION:  - Department of Internal Medicine, University of Michigan Medical Center, Ann Arbor, Mich., USA.

RESUMEN / SUMMARY:  - Pancreatic neuroendocrine tumors (PNETs) are relatively rare tumors that arise in the endocrine cells of the pancreas. Historically, somatostatin analogues have been used in this disease primarily for symptom control and, to a limited extent, disease stability. More recently, sunitinib and everolimus have been approved for advanced stage PNETs based on a survival benefit. However, both agents have a <10% actual response rate and cause nontrivial side effect profiles that limit duration of therapy. In locally advanced disease, there is a paucity of data to support an optimal neoadjuvant approach with the expectation of down-staging to allow for curative resection. We describe in this case a young woman who was successfully down-staged using a chemotherapy regimen of capecitabine and temozolomide with minimal toxicity.

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[226]

TÍTULO / TITLE:  - Non-invasive prenatal diagnosis of multiple endocrine neoplasia type 2A using COLD-PCR combined with HRM genotyping analysis from maternal serum.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - PLoS One. 2012;7(12):e51024. doi: 10.1371/journal.pone.0051024. Epub 2012 Dec 7.

            ●● Enlace al texto completo (gratuito o de pago) 1371/journal.pone.0051024

AUTORES / AUTHORS:  - Macher HC; Martinez-Broca MA; Rubio-Calvo A; Leon-Garcia C; Conde-Sanchez M; Costa A; Navarro E; Guerrero JM

INSTITUCIÓN / INSTITUTION:  - Department of Clinical Biochemistry, The Virgen del Rocio University Hospital (IBiS/CSIC/SAS/University of Seville), Seville, España.

RESUMEN / SUMMARY:  - The multiple endocrine neoplasia type 2A (MEN2A) is a monogenic disorder characterized by an autosomal dominant pattern of inheritance which is characterized by high risk of medullary thyroid carcinoma in all mutation carriers. Although this disorder is classified as a rare disease, the patients affected have a low life quality and a very expensive and continuous treatment. At present, MEN2A is diagnosed by gene sequencing after birth, thus trying to start an early treatment and by reduction of morbidity and mortality. We first evaluated the presence of MEN2A mutation (C634Y) in serum of 25 patients, previously diagnosed by sequencing in peripheral blood leucocytes, using HRM genotyping analysis. In a second step, we used a COLD-PCR approach followed by HRM genotyping analysis for non-invasive prenatal diagnosis of a pregnant woman carrying a fetus with a C634Y mutation. HRM analysis revealed differences in melting curve shapes that correlated with patients diagnosed for MEN2A by gene sequencing analysis with 100% accuracy. Moreover, the pregnant woman carrying the fetus with the C634Y mutation revealed a melting curve shape in agreement with the positive controls in the COLD-PCR study. The mutation was confirmed by sequencing of the COLD-PCR amplification product. In conclusion, we have established a HRM analysis in serum samples as a new primary diagnosis method suitable for the detection of C634Y mutations in MEN2A patients. Simultaneously,  we have applied the increase of sensitivity of COLD-PCR assay approach combined with HRM analysis for the non-invasive prenatal diagnosis of C634Y fetal mutations using pregnant women serum.

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[227]

TÍTULO / TITLE:  - Necrolytic migratory erythema associated with glucagonoma syndrome diagnosed by (68) Ga-DOTANOC PET-CT.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Asia Pac J Clin Oncol. 2012 Dec 26. doi: 10.1111/ajco.12048.

            ●● Enlace al texto completo (gratuito o de pago) 1111/ajco.12048

AUTORES / AUTHORS:  - Sahoo MK; Gupta S; Singh I; Pahwa S; Durgapal P; Bal CS

INSTITUCIÓN / INSTITUTION:  - Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India.

RESUMEN / SUMMARY:  - Necrolytic migratory erythema (NME) is a rare dermatological condition which presents a diagnostic challenge. Repeated negative skin biopsies and non-detection of any pancreatic tumor in conventional imaging modalities like a computed tomography (CT) scan and ultrasonogram (USG) make the diagnosis more difficult. By the time the diagnosis is made, the patient usually presents with metastasis. We present a rare case of difficult to diagnose NME, as repeated skin biopsies and conventional imaging modalities like CT and USG could not detect the underlying glucagonoma. A (68) Ga-DOTANOC positron emission tomography PET-CT was able to detect the underlying cause of NME as glucagonoma of the pancreas and the same investigation confirmed the absence of any metastasis elsewhere in the body. The tumor was excised and patient dramatically improved, and all skin lesions disappeared.

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[228]

TÍTULO / TITLE:  - Paraganglioma with unusual presentation in parotid gland: A diagnostic dilemma in fine needle aspiration.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Cytojournal. 2012;9:26. doi: 10.4103/1742-6413.105119. Epub 2012 Dec 27.

            ●● Enlace al texto completo (gratuito o de pago) 4103/1742-6413.105119

AUTORES / AUTHORS:  - Vora AA; Lai CK; Rao JY; Apple SK; Moatamed NA

INSTITUCIÓN / INSTITUTION:  - Address: Department of Pathology and Laboratory Medicine, David Geffen School of  Medicine at UCLA, Los Angeles, California, USA.

RESUMEN / SUMMARY:  - Paragangliomas (PGLs) are uncommon tumors. Although PGLs are known to occur in the head and neck region, especially the carotid body, middle ear, and larynx, involvement of the parotid glands has not been reported. In this article, we report the fine needle aspiration features of tumor in an unusual location, presenting as a parotid gland mass, submitted to pathology for initial diagnosis. The clinical presentation, cytomorphology, and the immunohistochemical features for the diagnosis are described. To our knowledge, this is the first case of paraganglioma of the parotid gland reported in the literature.

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[229]

TÍTULO / TITLE:  - ROS Production Is Essential for the Apoptotic Function of E2F1 in Pheochromocytoma and Neuroblastoma Cell Lines.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - PLoS One. 2012;7(12):e51544. doi: 10.1371/journal.pone.0051544. Epub 2012 Dec 12.

            ●● Enlace al texto completo (gratuito o de pago) 1371/journal.pone.0051544

AUTORES / AUTHORS:  - Espada L; Meo-Evoli N; Sancho P; Real S; Fabregat I; Ambrosio S; Tauler A

INSTITUCIÓN / INSTITUTION:  - Departament de Bioquimica i Biologia Molecular, Facultat de Farmacia. Universitat de Barcelona, Barcelona, Catalunya, España.

RESUMEN / SUMMARY:  - In this study we demonstrate that accumulation of reactive oxygen species (ROS) is essential for E2F1 mediated apoptosis in ER-E2F1 PC12 pheochromocytoma, and SH-SY5Y and SK-N-JD neuroblastoma stable cell lines. In these cells, the ER-E2F1  fusion protein is expressed in the cytosol; the addition of 4-hydroxytamoxifen (OHT) induces its translocation to the nucleus and activation of E2F1target genes. Previously we demonstrated that, in ER-E2F1 PC12 cells, OHT treatment induced apoptosis through activation of caspase-3. Here we show that caspase-8 activity did not change upon treatment with OHT. Moreover, over-expression of Bcl-xL arrested OHT-induced apoptosis; by contrast, over-expression of c-FLIP, did not have any effect on OHT-induced apoptosis. OHT addition induces BimL expression, its translocation to mitochondria and activation of Bax, which is paralleled by diminished mitochondrial enrichment of Bcl-xL. Treatment with a Bax-inhibitory peptide reduced OHT-induced apoptosis. These results point out the essential role of mitochondria on the apoptotic process driven by E2F1. ROS accumulation followed E2F1 induction and treatment with the antioxidant N-acetylcysteine, inhibited E2F1-induced Bax translocation to mitochondria and subsequent apoptosis. The role of ROS in mediating OHT-induced apoptosis was also studied in two neuroblastoma cell lines, SH-SY5Y and SK-N-JD. In SH-SY5Y cells, activation of E2F1 by the addition of OHT induced ROS production and apoptosis, whereas over-expression of E2F1 in SK-N-JD cells failed to induce either response. Transcriptional profiling revealed that many of the genes responsible for scavenging ROS were down-regulated following E2F1-induction in SH-SY5Y, but not in SK-N-JD cells. Finally, inhibition of GSK3beta blocked ROS production, Bax activation and the down regulation of ROS scavenging genes. These findings provide an explanation for the apparent contradictory role of E2F1 as an apoptotic agent versus a cell cycle activator.

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[230]

TÍTULO / TITLE:  - Insulinoma causing liver metastases 15 years after initial surgery, accompanied by glomerulonephritis.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Case Report Endocrinol. 2012;2012:168671. doi: 10.1155/2012/168671. Epub 2012 Dec 3.

            ●● Enlace al texto completo (gratuito o de pago) 1155/2012/168671

AUTORES / AUTHORS:  - Janez A

INSTITUCIÓN / INSTITUTION:  - Department of Endocrinology, Diabetes and Metabolic Diseases, University Medical  Center Ljubljana, Zaloska 7, 1000 Ljubljana, Slovenia.

RESUMEN / SUMMARY:  - Insulinoma is a rare pancreatic endocrine tumor that is typically sporadic, solitary, and less than 2 cm in diameter. Ninety percent of all insulinomas are benign. Up to 10 percent are malignant and are usually larger in size. We report  a case of an unusual, not yet described association of two diseases: a malignant  pancreatic insulinoma recurred as a multiple liver metastasis 15 years after the  initial complete enucleation of a primary tumor with a histomorphological fairly  benign outlook, accompanied by ANCA positive crescentic glomerulonephritis.

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[231]

TÍTULO / TITLE:  - Molecular heterogeneity in malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Hum Genomics. 2012 Sep 4;6:18. doi: 10.1186/1479-7364-6-18.

            ●● Enlace al texto completo (gratuito o de pago) 1186/1479-7364-6-18

AUTORES / AUTHORS:  - Thomas L; Mautner VF; Cooper DN; Upadhyaya M

INSTITUCIÓN / INSTITUTION:  - Institute of Medical Genetics, School of Medicine, Cardiff University, Cardiff CF14 4XN, UK.

RESUMEN / SUMMARY:  - Neurofibromatosis type-1 (NF1), resulting from NF1 gene loss of function, is characterized by an increased risk of developing benign and malignant peripheral  nerve sheath tumors (MPNSTs). Whereas the cellular heterogeneity of NF1-associated tumors has been well studied, the molecular heterogeneity of MPNSTs is still poorly understood. Mutational heterogeneity within these malignant tumors greatly complicates the study of the underlying mechanisms of tumorigenesis. We have explored this molecular heterogeneity by performing loss of heterozygosity (LOH) analysis of the NF1, TP53, RB1, PTEN, and CDKN2A genes on sections of 10 MPNSTs derived from 10 unrelated NF1 patients. LOH data for the TP53 gene was found to correlate with the results of p53 immunohistochemical analysis in the same tumor sections. Further, approximately 70% of MPNSTs were found to display intra-tumoral molecular heterogeneity as evidenced by differences in the level of LOH between different sections of the same tumor samples. This study constitutes the first systematic analysis of molecular heterogeneity within MPNSTs derived from NF1 patients. Appreciation of the existence of molecular heterogeneity in NF1-associated tumors is important not only for optimizing somatic mutation detection, but also for understanding the mechanisms of NF1 tumorigenesis, a prerequisite for the development of specifically targeted cancer therapeutics.

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[232]

TÍTULO / TITLE:  - Surgical excision with left atrial reconstruction of a primary functioning retrocardiac paraganglioma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Cardiothorac Surg. 2013 Jan 29;8(1):22.

            ●● Enlace al texto completo (gratuito o de pago) 1186/1749-8090-8-22

AUTORES / AUTHORS:  - Lopez MT; Gonzalez SG; Garcia ES; Romero SG; de Loma JG

RESUMEN / SUMMARY:  - ABSTRACT: About 2% of all paragangliomas are located in the chest, and a few have been described to be found in the heart. Primary cardiac paragangliomas are extremely uncommon tumors and surgical experience with this neoplasm is limited.  Treatment strategies described in the literature have included simple excision, excision with reconstruction, autotransplantation after excision of the tumor and even orthotopic cardiac transplantation, depending on the extent of disease. A primary retrocardiac paraganglioma catecholamine-productive was identified in an  asymptomatic 49-year old female associated to familial pheochromocytoma-paraganglioma syndrome caused by germline mutation of the gen which codifies for the subunit B of succinate dehydrogenase enzyme (SDHB). The neoplasm was surgically excised from the posterior surface of the left atrium via median sternotomy using cardiopulmonary bypass. Direct ligation of feeding vessels of the tumor along with left atrial reinforcement using a pericardial patch was performed. The post-operative course was uneventful, with normalization of catecholamine secretion and no recurrence at three-month follow-up. We review  the current literature about this exceptional cardiac tumor, pathophysiological conditions and options for surgical management.

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[233]

TÍTULO / TITLE:  - Poorly differentiated neuroendocrine carcinoma in a perigastric lymph node from an unknown primary site.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Cancer Res. Acceso gratuito al texto completo a partir de 1 año de la fecha de publicación.

            ●● Enlace a la Editora de la Revista http://cancerres.aacrjournals.org/ 

            ●● Cita: Cancer Research: <> Treat. 2012 Dec;44(4):271-4. doi: 10.4143/crt.2012.44.4.271. Epub 2012 Dec 31.

            ●● Enlace al texto completo (gratuito o de pago) 4143/crt.2012.44.4.271

AUTORES / AUTHORS:  - Lee HS; Han HS; Lim SN; Jeon HJ; Lee HC; Lee OJ; Yun HY; Lee KH; Kim ST

INSTITUCIÓN / INSTITUTION:  - Department of Internal Medicine, Chungbuk National University College of Medicine, Cheongju, Korea.

RESUMEN / SUMMARY:  - Neuroendocrine carcinomas from an unknown primary site are uncommon. The authors  report on a case of neuroendocrine carcinoma in a perigastric lymph node (LN) with no primary site. A 52-year-old male patient with early gastric adenocarcinoma underwent treatment by endoscopic submucosal dissection, and, six  months later, findings on a computed tomographic scan of the abdomen revealed a LN enlargement measuring 2.0 cm in the perigastric region. The patient underwent  subtotal gastrectomy and regional LN dissection under a suggestive preoperative diagnosis of gastric adenocarcinoma with LN metastasis. However, microscopically, no residual tumor was found in the stomach, and the perigastric LN showed poorly  differentiated neuroendocrine carcinoma (PDNEC). After an extensive workup, no primary site was identified. The patient also received four cycles of etoposide and cisplatin. Despite its extremely rare incidence, this case suggests that PDNEC of an unknown primary site is limited to a single site, and that resection  should be considered in combination with chemotherapy.

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[234]

TÍTULO / TITLE:  - Preoperative embolization followed by surgical excision of a giant thymic carcinoid.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Interact Cardiovasc Thorac Surg. 2012 Dec 30.

            ●● Enlace al texto completo (gratuito o de pago) 1093/icvts/ivs535

AUTORES / AUTHORS:  - Zhou Y; Chen C; Bao M; Jiang G; Zhu Y

INSTITUCIÓN / INSTITUTION:  - Department of Thoracic Surgery, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China.

RESUMEN / SUMMARY:  - We report the case of a 35-year old woman with a giant thymic carcinoid of the left hemithorax. Enhanced computed tomography showed marked vascularization of the tumour, with an enlarged drainage vein. Endovascular embolization of the major feeding arteries of the tumour was performed preoperatively with good angiographic results. A left thoracotomy was performed the following day. Minimal bleeding was observed due to prior embolization. The patient made a rapid postoperative recovery and was discharged 8 days later.

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[235]

TÍTULO / TITLE:  - Pedunculated duodenal carcinoid successfully removed by endoscopic resection with endoloop.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Dig Dis. 2013 Jan 9. doi: 10.1111/1751-2980.12033.

            ●● Enlace al texto completo (gratuito o de pago) 1111/1751-2980.12033

AUTORES / AUTHORS:  - Kimura T; Muguruma N; Goji T; Okamoto K; Kitamura S; Yano H; Miyamoto H; Okahisa T; Sakashita N; Takayama T

INSTITUCIÓN / INSTITUTION:  - Department of Gastroenterology and Oncology, The University of Tokushima Graduate School, Tokushima, Japan.

RESUMEN / SUMMARY:  - Duodenal carcinoid is a rare tumor, the natural history of which has not yet been clearly clarified. Endoscopic resection is recommended for tumors of less than 1  cm in diameter, limited to submucosal layer and with a low incidence of lymph node or distant metastases,(1,2) although no consensus has been reached till now. Several techniques have been reported, including endoscopic submucosal dissection (ESD),(2) strip biopsy(3) and band ligation technique(4) . In this study, we reported a case of pedunculated duodenal carcinoid which was successfully removed by endoscopic resection with endoloop.

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[236]

TÍTULO / TITLE:  - High (18)F-FDG uptake in sporadic paraganglioma of the retroperitoneum may be related to intra-tumor haemorrhage and macrophages.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Hell J Nucl Med. 2012 Sep-Dec;15(3):261.

AUTORES / AUTHORS:  - Kaida H; Kurata S; Kawahara A; Hiromatsu Y; Kage M; Ishibashi M

INSTITUCIÓN / INSTITUTION:  - Division of Nuclear Medicine, PET Center, Kurume University, School of Medicine,  Fukuoka, Japan. hayato@med.kurume-u.ac.jp

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[237]

TÍTULO / TITLE:  - Laparoscopic approach for duodenal carcinoid.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Wideochir Inne Tech Malo Inwazyjne. 2012 Jun;7(2):140-3. doi: 10.5114/wiitm.2011.25778. Epub 2011 Nov 20.

            ●● Enlace al texto completo (gratuito o de pago) 5114/wiitm.2011.25778

AUTORES / AUTHORS:  - Gunkova P; Gunka I; Dostalik J; Martinek L; Mazur M

INSTITUCIÓN / INSTITUTION:  - Department of Surgical Studies, Faculty of Medicine, University of Ostrava, Czech Republic.

RESUMEN / SUMMARY:  - Carcinoids are a heterogeneous group of neuroendocrine tumours. Duodenal localization is associated with relatively benign behaviour of the tumour, slow progression, low metastatic potential, and absence of endocrine activity. Type and extent of surgery depend on size and staging of the tumour. In this article,  we present a case study of a 27-year-old female patient with well-differentiated  carcinoid in the second part of the duodenum. The tumour of a size 5 mm x 3 mm x  2 mm manifested with abdominal symptomatology and did not invade the muscularis propria. Scintigraphy did not demonstrate other pathological deposits of somatostatin receptors. The wedge resection of the duodenal wall was performed laparoscopically with perioperative endoscopic localization and marking of the tumour. Uneventful operation and postoperative course presents a contribution of  miniinvasive surgery of specific duodenal lesions.

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[238]

TÍTULO / TITLE:  - Neuroendocrine tumor of the gallbladder.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Arch Iran Med. 2013 Feb;16(2):123-5. doi: 013162/AIM.0014.

AUTORES / AUTHORS:  - Elahi F; Ahmadzadeh A; Yadollahzadeh M; Hassanpour K; Babaei M

INSTITUCIÓN / INSTITUTION:  - Hematology and Oncology Research Center, Vali-Asr Hospital, Tehran University of  Medical Science, Tehran, Iran. ahmad.ahmadzadeh@gmail.com.

RESUMEN / SUMMARY:  - Neuroendocrine tumors (NET) arise from neuroendocrine cells and are an exceedingly rare malignancy in the gallbladder. In this case report, a 52-year-old woman with complaints of episodic abdominal pain for two months prior was admitted to our hospital. She had no other signs and symptoms and her laboratory tests were within normal limits. Ultrasonography showed a broad-necked mass (26 x 12 mm) in the gallbladder for which she underwent laparoscopic cholecystectomy. The final pathological diagnosis was a high grade neuroendocrine carcinoma of the gallbladder with involvement of the lymph nodes and omentum. The patient received the chemotherapy regimens of gemcitabine plus cisplatin, followed by docetaxel plus sunitinib for her metastatic liver lesions. She also underwent radiofrequency ablation. Serial CT-scans revealed metastatic liver lesions that had decreased in size, with no significant improvement. The patient  refused additional treatment and at 46 months, she was doing well with no complaints of any pain, disease recurrence, or metastatic progression.

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[239]

TÍTULO / TITLE:  - Primary hepatic gastrinoma as an unusual manifestation of zollinger-ellison syndrome.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Case Rep Gastroenterol. 2012 Sep;6(3):590-5. doi: 10.1159/000343157. Epub 2012 Sep 18.

            ●● Enlace al texto completo (gratuito o de pago) 1159/000343157

AUTORES / AUTHORS:  - Naoe H; Iwasaki H; Kawasaki T; Ozaki T; Tsutsumi H; Okuda A; Konoe T; Nonaka K; Kaku E; Shono T; Yokomine K; Sakurai K; Iyama K; Hirota M; Sasaki Y

INSTITUCIÓN / INSTITUTION:  - Departments of Gastroenterology and Hepatology.

RESUMEN / SUMMARY:  - We report a rare case of primary hepatic gastrinoma. A 77-year-old woman exhibited continuous watery diarrhea for 8 months and weight loss. Bacterial cultures of the stools were negative and colonoscopy revealed no abnormalities. Esophagogastroduodenoscopy showed severe reflux esophagitis and multiple duodenal erosions. Computed tomography and magnetic resonance imaging detected two solid masses measuring <2 cm in diameter in the right lobe of the non-cirrhotic liver.  Microscopically, the tumor was consistent with neuroendocrine tumor (grade 2) with abundant gastrin-immunoreactive cells. Endoscopic ultrasound detected no other alternative primary source of an endocrine tumor. The serum gastrin levels  exceeded 40,000 pg/ml in the absence of H(2) receptor antagonist and proton pump  inhibitor administrations. Based on an arterial stimulation and venous sampling test, the patient was diagnosed as primary gastrinoma of the liver. Our findings  demonstrated the presence of Zollinger-Ellison syndrome in a patient who was subsequently cured by surgical resection of the liver tumors.

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[240]

TÍTULO / TITLE:  - Paraganglioma of the bladder.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Clin Adv Hematol Oncol. 2012 Dec;10(12):839-41.

AUTORES / AUTHORS:  - Jansen R; Zaslau S

INSTITUCIÓN / INSTITUTION:  - West Virginia University, Division of Urology, Morgantown, West Virginia.

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[241]

TÍTULO / TITLE:  - Malignant paraganglioma of the urinary bladder in a 45-year-old woman.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Clin Adv Hematol Oncol. 2012 Dec;10(12):836-9.

AUTORES / AUTHORS:  - Palla AR; Hogan T; Singh S

INSTITUCIÓN / INSTITUTION:  - Danbury Hospital, Danbury, CT.

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[242]

- CASTELLANO -

TÍTULO / TITLE:Cromogranina A y tumores neuroendocrinos.

TÍTULO / TITLE:  - Chromogranin A and neuroendocrine tumors.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Endocrinol Nutr. 2012 Dec 25. pii: S1575-0922(12)00322-1. doi: 10.1016/j.endonu.2012.10.003.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.endonu.2012.10.003

AUTORES / AUTHORS:  - Diaz Perez JA; Curras Freixes M

INSTITUCIÓN / INSTITUTION:  - Servicio de Endocrinologia y Nutricion, Hospital Clinico San Carlos, Madrid, España. Electronic address: joseangeldiaz@mixmail.com.

RESUMEN / SUMMARY:  - Chromogranin A (CgA) is the most abundant granin in gastroenteropancreatic neuroendocrine tumors (GEP-NETs). As a tumor marker is moderately sensitive and nonspecific. Despite the limitations of testing methods, which require careful interpretation, especially in the case of gastrinomas, patients treated with somatostatin analogues, and poorly differentiated tumors, it is the best tumor marker in GEP-NETs and may be of value in other tumors with neuroendocrine differentiation. CgA may be used as a marker in blood or tissue samples through immunohistochemical techniques. CgA levels correlate with tumor burden and extension and may be used for diagnosis and monitoring of GEP-NETs, especially midgut carcinoids and endocrine pancreatic tumors. It is also useful as a prognostic marker for detection of recurrence and monitoring of response to different treatments.

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[243]

TÍTULO / TITLE:  - Concomitant intraductal papillary mucinous neoplasm and neuroendocrine tumor of the pancreas.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Oncol Lett. 2013 Jan;5(1):63-67. Epub 2012 Oct 3.

            ●● Enlace al texto completo (gratuito o de pago) 3892/ol.2012.952

AUTORES / AUTHORS:  - Ishida M; Shiomi H; Naka S; Tani T; Okabe H

INSTITUCIÓN / INSTITUTION:  - Department of Clinical Laboratory Medicine and Division of Diagnostic Pathology;

RESUMEN / SUMMARY:  - Intraductal papillary mucinous neoplasm (IPMN) and neuroendocrine tumor (NET) of  the pancreas are rare tumors and their association is not expected to be frequent. However, certain studies have suggested that the concomitant occurence  of these tumors may be more frequent than previously thought. In the current study, we describe a case of concomitant occurrence of IPMN and NET of the pancreas and review the clinicopathological features of previously published cases and the current one. A 68-year-old female was incidentally found to have dilatation of the main pancreatic duct. A distal pancreatectomy was performed under the clinical diagnosis of IPMN. Histopathological analysis revealed concomitant IPMN (low-grade) and NET G1 of the pancreas. Review of the clinicopathological features of the 15 previously reported cases of concomitant IPMN and NET of the pancreas as well as the present one indicated that: i) this condition mainly affects middle-aged females; ii) the main symptom is abdominal or back pain, or no symptoms; iii) a hormone production symptom was observed in only one case; iv) the most common degree of dysplasia of IPMN is low grade; and  v) the size of the NET is not particularly large (average 15.1 mm), although the  clinical behavior is not always indolent (metastasis was observed in 3 cases). It is well known that IPMNs are associated with a high incidence of extrapancreatic  malignancies, including colorectal and gastric carcinomas. Concomitant pancreatic NET and extrapancreatic malignancies may occur, therefore, systemic surveillance  of extrapancreatic neoplasms and detection of concomitant NETs of the pancreas are necessary for patients with IPMN.

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[244]

TÍTULO / TITLE:  - An unusual presentation of a carcinoid tumor of the common bile duct.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - JOP. 2013 Jan 10;14(1):85-7. doi: 10.6092/1590-8577/1232.

AUTORES / AUTHORS:  - Jethava A; Muralidharan V; Mesologites T; Stoica-Mustafa E; Dasanu CA

INSTITUCIÓN / INSTITUTION:  - Department of Hematology-Oncology, Saint Francis Hospital and Medical Center. Hartford, CT, USA. c_dasanu@yahoo.com.

RESUMEN / SUMMARY:  - CONTEXT: Carcinoid tumors arising from the bile ducts account for only a small fraction of biliary tract cancers. CASE REPORT: We report herein a 42-year-old man with a carcinoid tumor of the common bile duct. He presented with abdominal pain, bloating and dyspepsia. Clinicolaboratory and imaging studies suggested a probable obstructive common bile duct lesion. The patient underwent an endoscopic retrograde cholangiopancreatography with a stent placement in view of common bile duct decompression. Persistence of symptoms prompted a laparotomy and pancreaticoduodenectomy that revealed a well-differentiated carcinoid tumor originating in the common bile duct. CONCLUSION: Clinician’s familiarity with the unusual sites of origin of neuroendocrine tumors and/or atypical presentation of  such tumors may facilitate their early recognition and allow for a timely intervention.

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[245]

TÍTULO / TITLE:  - Mixed neuroendocrine tumor of the common bile duct.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - JOP. 2013 Jan 10;14(1):71-3. doi: 10.6092/1590-8577/1225.

AUTORES / AUTHORS:  - Linder R; Dorfman T; Ben-Ishay O; Kakiashvili E; Velodavsky E; Kluger Y

INSTITUCIÓN / INSTITUTION:  - Division of Surgical Oncology, Department of General Surgery, Rambam Health Care  Center. Haifa, Israel. o_ben-ishay@rambam.health.gov.il.

RESUMEN / SUMMARY:  - CONTEXT: Mixed adenoendocrine tumors of the extrahepatic bile ducts are exceedingly rare and most of those who are rarely diagnosed are adenocarcinomas.  Neuroendorine tumors accounts for only 0.2-2%. CASE REPORT: We report a case of mixed adenoneuroendo-carcinoma of the common bile duct in an 82-year-old male. CONCLUSION: Clinical experience suggests that the neuroendocrine component of mixed tumors behave more aggressively than the regular biliary adenocarcinoma component. This clinical behavior may have an important role in the management of this clinical entity.

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[246]

TÍTULO / TITLE:  - Coexistence of pheochromocytoma/praganglioma and renal artery stenosis.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Indian J Endocrinol Metab. 2012 Nov;16(6):1009-11. doi: 10.4103/2230-8210.103022.

            ●● Enlace al texto completo (gratuito o de pago) 4103/2230-8210.103022

AUTORES / AUTHORS:  - Sarathi V; Bandgar T; Lila AR; Deshpande AA; Dalvi AN; Patwardhan S; Shah NS

INSTITUCIÓN / INSTITUTION:  - Department of Endocrinology, Seth G S Medical College, Parel, Mumbai, Maharashtra, India.

RESUMEN / SUMMARY:  - Renal artery stenosis (RAS) often coexists with pheochromocytoma (Pheo)/paraganglioma (PGL) and often alters the management of patients with Pheo/PGL. We have studied the prevalence of RAS in our Pheo/PGL patients. The study included 70 consecutive, histopathologically proven Pheo/PGL patients from  a tertiary health care center. In 60 patients, tumors were limited to adrenal glands (54 unilateral and 6 bilateral) while other 10 patients had extra-adrenal  abdominal tumors. Five patients had RAS with an overall prevalence of 14%. Only two out of 60 patients with Pheo had RAS with a low prevalence of 3.3% while three out of 10 patients with extra-adrenal abdominal PGL had RAS with a prevalence of 30%. To conclude, RAS commonly coexists with Pheo/PGL, more often with extra-adrenal PGL.

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[247]

TÍTULO / TITLE:  - Coexistence of pheochromocytoma with uncommon vascular lesions.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Indian J Endocrinol Metab. 2012 Nov;16(6):962-71. doi: 10.4103/2230-8210.103000.

            ●● Enlace al texto completo (gratuito o de pago) 4103/2230-8210.103000

AUTORES / AUTHORS:  - Kota SK; Kota SK; Meher LK; Jammula S; Panda S; Modi KD

INSTITUCIÓN / INSTITUTION:  - Department of Endocrinology, Medwin Hospital, Hyderabad, Andhra Pradesh, India.

RESUMEN / SUMMARY:  - BACKGROUND: Pheochromocytoma/paragangliomas have been described to be associated  with rare vascular abnormalities like renal artery stenosis. Coexistence of physiologically significant renal artery lesions is a compounding factor that alters management and prognosis of pheochromocytoma patients. Apart from individual case reports, data on such association in Indian population is not available. The aim of this study is to find the nature and prevalence of associated vascular abnormalities. MATERIALS AND METHODS: From 1990 to 2010, a total of 50 patients were diagnosed with pheochromocytoma/paragangliomas. Hospital charts of these patients were reviewed retrospectively to identify those with unusual vascular abnormalities. Available literature was also reviewed. RESULTS: Of the 50 patients with pheochromocytoma, 7 (14%) had coexisting vascular lesions including renal artery stenosis in 4, aortoarteritis in 1, aortic aneurysm in 1 and inferior vena cava thrombosis in 1. Pheochromocytoma was adrenal in 42 and extra adrenal in 8. Laparoscopic adrenalectomy was done in the  patients. One patient with renal artery stenosis due to intimal fibrosis was subjected to percutaneous balloon angioplasty; the other three improved after adrenalectomy and lysis of fibrous adhesive bands. The patient with aortoarteritos was treated with oral steroids. Inferior vena cava thrombosis was  reversed with anticoagulants. The patient with abdominal aortic aneurysm was advised for annual follow-up on account of its size of 4.5 cm and asymptomatic presentation. CONCLUSION: There are multiple mechanisms that can lead to renal artery stenosis and other vascular abnormalities in a case of pheochromocytoma. A high index of suspicion is necessary to enable both entities to be diagnosed preoperatively and allow proper planning of surgical therapy. Incomplete diagnosis may lead to persistent hypertension postoperatively in a case of associated renal artery stenosis.

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[248]

TÍTULO / TITLE:  - Multiple endocrine neoplasia type 1 (MEN1).

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Asia Pac J Clin Oncol. 2012 Dec 26. doi: 10.1111/ajco.12046.

            ●● Enlace al texto completo (gratuito o de pago) 1111/ajco.12046

AUTORES / AUTHORS:  - Carroll RW

INSTITUCIÓN / INSTITUTION:  - Endocrine, Diabetes and Research Centre, Wellington Regional Hospital, Wellington, New Zealand.

RESUMEN / SUMMARY:  - Multiple endocrine neoplasia type 1 (MEN1) is inherited in an autosomal dominant  fashion and predisposes to the development of hyperplastic or neoplastic changes  in the parathyroid and pituitary glands and the endocrine pancreas, along with numerous other characteristic tumors and features. The management of each entity  differs to some degree from their sporadic counterparts, while the lack of a genotype-phenotype correlation requires lifelong clinical, biochemical and radiological screening for the development of new tumors. While the syndrome itself is relatively rare (a prevalence of 1-10/100 000), it is likely that health-care practitioners from numerous specialities will occasionally encounter  a patient with MEN1 and therefore a basic knowledge of the syndrome is important. In addition, many of the associated tumors are seen commonly in sporadic form, and a judicious policy is therefore required in deciding how thoroughly patients  who develop these tumors should be screened for MEN1. The current literature on MEN1 is reviewed and key learning points are suggested for the clinician.

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[249]

TÍTULO / TITLE:  - Pedunculated insulinoma on the anterior border of the head of the pancreas: An unusual location to be aware of.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Diagn Interv Imaging. 2013 Jan 23. pii: S2211-5684(12)00174-X. doi: 10.1016/j.diii.2012.04.025.

            ●● Enlace al texto completo (gratuito o de pago) 1016/j.diii.2012.04.025

AUTORES / AUTHORS:  - Sebbag-Sfez D; Berrod JL; Palazzo L; Zins M

INSTITUCIÓN / INSTITUTION:  - Department of Radiology and Digestive Surgery, fondation hopital Saint-Joseph, 185, rue Raymond-Losserand, 75014 Paris, France. Electronic address: delphinesebbag@gmail.com.

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[250]

TÍTULO / TITLE:  - Our experience with carotid body paragangliomas.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Prague Med Rep. 2012;113(4):262-70.

AUTORES / AUTHORS:  - Erdogan BB; Bora F; Altin G; Paksoy M

INSTITUCIÓN / INSTITUTION:  - Department of Otolaryngology, Dr. Lutfi Kirdar Kartal Education and Research Hospital, Istanbul, Turkey.

RESUMEN / SUMMARY:  - Carotid body paragangliomas (CBP) are rare neoplasms arising from the small chemoreceptor organ in the adventitia of the common carotid bifurcation. The aim  of this study is to present the diagnostic process, performed treatment and obtained results in patients with carotid body paragangliomas of the Department of Otolaryngology of Istanbul Education and Research Hospital between March 1997  and November 2008. Retrospective analysis was carried out, based on the medical documentation of 5 patients with carotid body paragangliomas (3 women and 2 men), age range 44 to 68 years with a mean of 59.6 years. Four of the patients were diagnosed and treated with Shamblin type II tumor, one of the patients with type  I. Physical examination, radiological evaluation, method of the treatment and post-treatment complications were studied. The most common and single symptom was nonspecific neck mass. Preoperative diagnostic evaluation consisted of a color duplex ultrasonography, computerized tomography with contrast enhancement, magnetic resonance imaging and digital subtraction angiography. In all patients with Shamblin type I and II, blunt dissection of the tumor was conducted smoothly in the subadventitial plane. Postoperative vagus nerve and hypoglossal nerve deficit were reported in one case. Carotid body paraganglioma excision has higher risk of cranial nerve paresis and carotid artery injury, so it requires careful handling and good surgical skills to ensure complete removal.

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[251]

TÍTULO / TITLE:  - Histopathology of gastrointestinal neuroendocrine neoplasms.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - Front Oncol. 2013;3:2. doi: 10.3389/fonc.2013.00002. Epub 2013 Jan 22.

            ●● Enlace al texto completo (gratuito o de pago) 3389/fonc.2013.00002

AUTORES / AUTHORS:  - Hirabayashi K; Zamboni G; Nishi T; Tanaka A; Kajiwara H; Nakamura N

INSTITUCIÓN / INSTITUTION:  - Department of Pathology, Ospedale Sacro Cuore Don Calabria Negrar, Verona, Italy  ; Department of Pathology, Tokai University School of Medicine Isehara, Kanagawa, Japan.

RESUMEN / SUMMARY:  - Gastrointestinal neuroendocrine neoplasms (GI-NENs) arise from neuroendocrine cells distributed mainly in the mucosa and submucosa of the gastrointestinal tract. In 2010, the World Health Organization (WHO) classification of NENs of the digestive system was changed, categorizing these tumors as grade 1 neuroendocrine tumor (NET), grade-2NET, neuroendocrine carcinoma (large- or small-cell type), or mixed adenoneuroendocrine carcinoma (MANEC). Such a classification is based on the Ki-67 index and mitotic count in histological material. For the accurate pathological diagnosis and grading of NENs, it is important to clearly recognize  the characteristic histological features of GI-NENs and to understand the correct method of counting Ki-67 and mitoses. In this review, we focus on the histopathological features of GI-NENs, particularly regarding biopsy and cytological diagnoses, neuroendocrine markers, genetic and molecular features, and the evaluation of the Ki-67 index and mitotic count. In addition, we will address the histological features of GI-NEN in specific organs.

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[252]

TÍTULO / TITLE:  - Functioning glucagonoma associated with primary hyperparathyroidism: multiple endocrine neoplasia type 1 or incidental association?

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - BMC Cancer. 2012 Dec 22;12:614. doi: 10.1186/1471-2407-12-614.

            ●● Enlace al texto completo (gratuito o de pago) 1186/1471-2407-12-614

AUTORES / AUTHORS:  - Erdas E; Aste N; Pilloni L; Nicolosi A; Licheri S; Cappai A; Mastinu M; Cetani F; Pardi E; Mariotti S; Pomata M

INSTITUCIÓN / INSTITUTION:  - General Surgery Unit, Department of Surgical Sciences, San Giovanni di Dio Hospital, University of Cagliari, Cagliari, Italy. enricoerdas@medicina.unica.it.

RESUMEN / SUMMARY:  - ABSTRACT: BACKGROUND: Diagnosis of multiple endocrine neoplasia type 1 (MEN1) is  commonly based on clinical criteria, and confirmed by genetic testing. In patients without known MEN1-related germline mutations, the possibility of a casual association between two or more endocrine tumors cannot be excluded and subsequent management may be difficult to plan. We describe a very uncommon case  of functioning glucagonoma associated with primary hyperparathyroidism (pHPT) in  which genetic testing failed to detect germline mutations of MEN-1 and other known genes responsible for MEN1. CASE PRESENTATION: The patient, a 65-year old woman, had been suffering for more than 1 year from weakness, progressive weight  loss, angular cheilitis, glossitis and, more recently, skin rashes on the perineum, perioral skin and groin folds. After multidisciplinary investigations,  functioning glucagonoma and asymptomatic pHPT were diagnosed and, since family history was negative, sporadic MEN1 was suspected. However, genetic testing revealed neither MEN-1 nor other gene mutations responsible for rarer cases of MEN1 (CDKN1B/p27 and other cyclin-dependent kinase inhibitor genes CDKN1A/p15, CDKN2C/p18, CDKN2B/p21). The patient underwent distal splenopancreatectomy and at the 4-month follow-up she showed complete remission of symptoms. Six months later, a thyroid nodule, suspected to be a malignant neoplasia, and two hyperfunctioning parathyroid glands were detected respectively by ultrasound with fine needle aspiration cytology and 99mTc-sestamibi scan with SPECT acquisition.  Total thyroidectomy was performed, whereas selective parathyroidectomy was preferred to a more extensive procedure because the diagnosis of MEN1 was not supported by genetic analysis and intraoperative intact parathyroid hormone had revealed “adenoma-like” kinetics after the second parathyroid resection. Thirty-nine and 25 months after respectively the first and the second operation,  the patient is well and shows no signs or symptoms of recurrence. CONCLUSIONS: Despite well-defined diagnostic criteria and guidelines, diagnosis of MEN1 can still be challenging. When diagnosis is doubtful, appropriate management may be difficult to establish.

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[253]

TÍTULO / TITLE:  - Incidental carcinoid tumor next to vascular malformation.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - J Bronchology Interv Pulmonol. 2013 Jan;20(1):77-9. doi: 10.1097/LBR.0b013e31827ccec0.

            ●● Enlace al texto completo (gratuito o de pago) 1097/LBR.0b013e31827ccec0

AUTORES / AUTHORS:  - Casimire T; Cutaia M; Morgan D; Dubey G; Al-Ajam MR

INSTITUCIÓN / INSTITUTION:  - *State University of New York Downstate Medical Center daggerDepartment of Veterans Administration New York Harbor Healthcare System the Brooklyn Campus, Brooklyn, NY.

RESUMEN / SUMMARY:  - Dedicated linear endobronchial ultrasound (EBUS) is an established modality in sampling central pulmonary lesions, mediastinal and hilar lymph nodes to diagnose and stage lung malignancies as well as evaluate a number of inflammatory conditions. In addition, power Doppler modality of EBUS allows for vascular evaluation. We describe a case where EBUS visualized a submucosal lesion within a vascular malformation, which was eventually proven to be a carcinoid tumor. Computed tomographic scan of the chest failed to differentiate the 2 structures and bronchoscopy showed no endobronchial component. This case underscores the added diagnostic capabilities of endobronchial Doppler ultrasonography. We suggest considering EBUS in investigating central opacities associated with vascular features within the lung parenchyma when technically feasible.

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[254]

TÍTULO / TITLE:  - MSH2 and CXCR4 involvement in malignant VIPoma.

RESUMEN / SUMMARY:  - Enlace al Resumen / Link to its Summary

REVISTA / JOURNAL:  - World J Surg Oncol. 2012 Dec 11;10:264. doi: 10.1186/1477-7819-10-264.

            ●● Enlace al texto completo (gratuito o de pago) 1186/1477-7819-10-264

AUTORES / AUTHORS:  - Muller S; Kupka S; Konigsrainer I; Northoff H; Sotlar K; Bock T; Kandolf R; Traub F; Konigsrainer A; Zieker D

INSTITUCIÓN / INSTITUTION:  - Department of General, Visceral and Transplant Surgery, Tubingen, Germany. derek.zieker@med.uni-tuebingen.de.

RESUMEN / SUMMARY:  - ABSTRACT: BACKGROUND: Vasoactive intestinal polypeptide secreting tumors(VIPomas) are rare endocrine tumors of the pancreas with an estimated incidence of 0.1 per  million per year. The molecular mechanisms that mediate development of VIPomas are poorly investigated and require definition. METHODS: A genome- and gene expression analysis of specimens of a primary pancreatic VIPoma with hepatic metastases was performed. The primary tumor, the metastases, the corresponding healthy tissue of the liver, and the pancreas were compared with each other using oligonucleotide microarrays and loss of heterozygosity (LOH). RESULTS: The results revealed multiple LOH events and several differentially expressed genes.  Our finding of LOH and downregulation was conspicuous in the microarray analysis  for the mismatch repair gene MSH2 in the primary pancreatic VIPoma tumor, the hepatic metastasis but not in the corresponding healthy tissue. Further a strong  overexpression of the chemokine CXCR4 was detected in the hepatic metastases compared to its pancreatic primary. With a review of the literature we describe the molecular insights of metastatic development in VIPoma. CONCLUSION: In VIPoma, defects in the mismatch repair system especially in MSH2 may contribute to carcinogenesis, and increased CXCR4 may be associated with liver metastasis.

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