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Metastatic Potential and Survival of Duodenal and Pancreatic Tumors in Multiple Endocrine Neoplasia Type 1: A GTE and AFCE Cohort Study (Groupe d'étude des Tumeurs Endocrines and Association Francophone de Chirurgie Endocrinienne).

Published on Dec 21, 2018in Annals of Surgery9.476
· DOI :10.1097/SLA.0000000000003162
Sandrine Vinault5
Estimated H-index: 5
,
Anne-Sophie Mariet1
Estimated H-index: 1
+ 25 AuthorsPierre Goudet21
Estimated H-index: 21
Abstract
Objective:To assess the distant metastatic potential of duodeno-pancreatic neuroendocrine tumors (DP-NETs) in patients with MEN1, according to functional status and size.Summary Background Data:DP-NETs, with their numerous lesions and endocrine secretion-related symptoms, continue to be a medical ch
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  • Citations (8)
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#1Samira M. Sadowski (NIH: National Institutes of Health)H-Index: 18
#2Carolina R. C. Pieterman (UU: Utrecht University)H-Index: 10
Last. Gerlof D. Valk (UU: Utrecht University)H-Index: 31
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Metastatic duodenopancreatic neuro-endocrine tumors (dpNETs) are the most important disease-related cause of death in patients with multiple endocrine neoplasia type 1 (MEN1). Nonfunctioning pNETs (NF-pNETs) are highly prevalent in MEN1 and clinically heterogeneous. Therefore, management is controversial. Data on prognostic factors for risk stratification is limited. This systematic review aims to establish the current state of evidence regarding prognostic factors in MEN1-related NF-pNETs. We s...
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BACKGROUND: Insulinomas are found in 10-15 per cent of patients with multiple endocrine neoplasia type 1 (MEN1) and lead to life-threatening hypoglycaemia. Surgical outcome and the optimal surgical strategy for MEN1-related insulinoma are unknown. METHODS: Patients with MEN1-related insulinomas were identified in 46 centres in Europe and North America between 1990 and 2016. Insulinomas were considered localized if the lesion was in the pancreatic head or body/tail. Patients with pancreatic neuro...
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#1Anne CouvelardH-Index: 54
#2Jean-Yves Scoazec (Institut Gustave Roussy)H-Index: 63
Resume Environ 5 % des tumeurs neuroendocrines (TNE) gastro-entero-pancreatiques et thoraciques se developpent dans le cadre d’un syndrome de predisposition genetique hereditaire. Les deux syndromes les plus frequents sont : la neoplasie endocrinienne multiple de type 1 (NEM1), associee a un large spectre tumoral incluant des TNE duodenopancreatiques, thymiques et bronchiques, et le syndrome de von Hippel-Lindau (VHL), associe a des TNE pancreatiques. Deux autres syndromes presentent une faible ...
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#1Antongiulio Faggiano (Sapienza University of Rome)H-Index: 3
#2Roberta ModicaH-Index: 4
Last. Annamaria ColaoH-Index: 83
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PURPOSE: Pancreatic neuroendocrine tumors (pNETs) are frequent in multiple endocrine neoplasia type 1 (MEN1) syndrome. They are usually not surgically treated unless larger than 1 to 2 cm or a growth rate > 0.5 cm per year. Somatostatin analogues represent one of the main therapeutic options in pNETs, but they have never been prospectively investigated in MEN1-related pNETs. The aim of this study was to prospectively evaluate the effectiveness of lanreotide in patients with MEN1-related pNETs < ...
5 CitationsSource
#1Elisabeth Hain (Paris V: Paris Descartes University)H-Index: 1
Last. Sébastien Gaujoux (Paris V: Paris Descartes University)H-Index: 31
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ABSTRACTIntroduction: Neuroendocrine tumors of the pancreas (pNETs) represent only 1% to 2% of all pancreatic neoplasms. These tumors can be classified as functional or nonfunctional tumors; as spo...
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#1Lingaku Lee (NIH: National Institutes of Health)H-Index: 8
#2Tetsuhide Ito (International University of Health and Welfare)H-Index: 2
Last. Robert T. Jensen (NIH: National Institutes of Health)H-Index: 95
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ABSTRACTIntroduction: Recent advances in diagnostic modalities and therapeutic agents have raised the importance of prognostic factors in predicting overall survival, as well as predictive factors ...
2 CitationsSource
Abstract About 30% of patients with MEN1 develop a Zollinger-Ellison syndrome. Meanwhile it is well established that the causative gastrinomas are almost exclusively localized in the duodenum and not in the pancreas, MEN1 gastrinomas occur multicentric and are associated with hyperplastic gastrin cell lesions and tiny gastrin-producing micro tumors in contrast to sporadic duodenal gastrinomas. Regardless of the high prevalence of early lymphatic metastases, the survival is generally good with an...
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#1A. Sauvanet (Paris Diderot University)H-Index: 11
Abstract Natural history of gastroenteropancreatic (GEP) Neuroendocrine tumors (NETs) is better and better known so indications of surgery are presently selective. Surgical resection, but also endoscopic resection and observation, can be proposed for gastric NETs according to presentation, size and grade. For small bowel NETs, resection is frequently needed but should obtain the best compromise between radicality and postoperative functional disorders. Appendiceal NETs are frequently diagnosed b...
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