PMID- 31401809 OWN - NLM STAT- MEDLINE DCOM- 20191023 LR - 20210110 IS - 1096-9098 (Electronic) IS - 0022-4790 (Print) IS - 0022-4790 (Linking) VI - 120 IP - 6 DP - 2019 Nov TI - Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG). PG - 966-975 LID - 10.1002/jso.25667 [doi] AB - BACKGROUND AND OBJECTIVES: Gastrinomas are the most prevalent functioning neuroendocrine tumors (NET) in multiple endocrine neoplasia type 1 (MEN1). Guidelines suggest medical therapy in most patients, but surgery may be considered in a subgroup. Currently, factors to guide management are necessary. This population-based cohort study assessed prognostic factors of survival in patients with MEN1-related gastrinomas. METHODS: Patients with MEN1 having gastrinomas were identified in the Dutch MEN1 database from 1990 to 2014 based on fasting serum gastrin (FSG) levels and/or pathology. Predictors of overall survival were assessed using Cox regression. RESULTS: Sixty-three patients with gastrinoma (16% of the MEN1 population) were identified. Five- and 10-year overall survival rates were 83% and 65%, respectively. Prognostic factors associated with overall survival were initial FSG levels >/=20x upper limit of normal (ULN) (hazard ratio [HR], 6.2 [95% confidence interval, 1.7-23.0]), pancreatic NET >/=2 cm (HR 4.5; [1.5-13.1]), synchronous liver metastases (HR 8.9; [2.1-36.7]), gastroduodenoscopy suspicious for gastric NETs (HR 12.7; [1.4-115.6]), and multiple concurrent NETs (HR 5.9; [1.2-27.7]). CONCLUSION: Life expectancy of patients with MEN1 gastrinoma is reduced. FSG levels and pancreatic NETs >/=2 cm are prognostic factors. FSG levels might guide surveillance intensity, step-up to additional diagnostics, or provide arguments in selecting patients who might benefit from surgery. CI - (c) 2019 The Authors. Journal of Surgical Oncology published by Wiley Periodicals, Inc. FAU - van Beek, Dirk-Jan AU - van Beek DJ AUID- ORCID: 0000-0001-7834-8128 AD - Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Nell, Sjoerd AU - Nell S AD - Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Pieterman, Carolina R C AU - Pieterman CRC AD - Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - de Herder, Wouter W AU - de Herder WW AD - Department of Internal Medicine, Erasmus Medical Center, Rotterdam, The Netherlands. FAU - van de Ven, Annenienke C AU - van de Ven AC AD - Department of Endocrinology, Radboud University Medical Center, Nijmegen, The Netherlands. FAU - Dekkers, Olaf M AU - Dekkers OM AD - Departments of Endocrinology and Metabolism and Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands. FAU - van der Horst-Schrivers, Anouk N AU - van der Horst-Schrivers AN AD - Department of Endocrinology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands. FAU - Drent, Madeleine L AU - Drent ML AD - Department of Internal Medicine, Section of Endocrinology, Amsterdam UMC location, VU University Medical Center, Amsterdam, The Netherlands. FAU - Bisschop, Peter H AU - Bisschop PH AD - Department of Endocrinology and Metabolism, Amsterdam UMC location Academic Medical Center, Amsterdam, The Netherlands. FAU - Havekes, Bas AU - Havekes B AD - Department of Internal Medicine, Division of Endocrinology, Maastricht University Medical Center, Maastricht, The Netherlands. FAU - Borel Rinkes, Inne H M AU - Borel Rinkes IHM AD - Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Vriens, Menno R AU - Vriens MR AD - Department of Endocrine Surgical Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. FAU - Valk, Gerlof D AU - Valk GD AD - Department of Endocrine Oncology, University Medical Center Utrecht, Utrecht, The Netherlands. LA - eng GR - Ipsen Pharmaceutical/ PT - Journal Article DEP - 20190810 PL - United States TA - J Surg Oncol JT - Journal of surgical oncology JID - 0222643 RN - 0 (MEN1 protein, human) RN - 0 (Proto-Oncogene Proteins) RN - Gastro-enteropancreatic neuroendocrine tumor SB - IM MH - Cohort Studies MH - Female MH - Follow-Up Studies MH - Gastrinoma/metabolism/*mortality/pathology/surgery MH - Humans MH - Intestinal Neoplasms/metabolism/*mortality/pathology/surgery MH - Liver Neoplasms/metabolism/*mortality/secondary/surgery MH - Male MH - Middle Aged MH - Netherlands MH - Neuroendocrine Tumors/metabolism/*mortality/pathology/surgery MH - Pancreatic Neoplasms/metabolism/*mortality/pathology/surgery MH - Prognosis MH - Proto-Oncogene Proteins/*metabolism MH - Stomach Neoplasms/metabolism/*mortality/pathology/surgery MH - Survival Rate PMC - PMC6852496 OTO - NOTNLM OT - Zollinger-Ellison syndrome OT - multiple endocrine neoplasia type 1 OT - neuroendocrine tumor OT - oncology COIS- There is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported. EDAT- 2019/08/12 06:00 MHDA- 2019/10/24 06:00 PMCR- 2019/11/13 CRDT- 2019/08/12 06:00 PHST- 2019/07/23 00:00 [received] PHST- 2019/07/28 00:00 [accepted] PHST- 2019/08/12 06:00 [pubmed] PHST- 2019/10/24 06:00 [medline] PHST- 2019/08/12 06:00 [entrez] PHST- 2019/11/13 00:00 [pmc-release] AID - JSO25667 [pii] AID - 10.1002/jso.25667 [doi] PST - ppublish SO - J Surg Oncol. 2019 Nov;120(6):966-975. doi: 10.1002/jso.25667. Epub 2019 Aug 10.