Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG)

Dirk-Jan van Beek, Sjoerd Nell, Carolina R. C. Pieterman, Wouter W. de Herder, Annenienke C. van de Ven, Olaf M. Dekkers, Anouk N. van der Horst-Schrivers, Madeleine L. Drent, Peter H. Bisschop, Bas Havekes, Inne H. M. Borel Rinkes, Menno R. Vriens, Gerlof D. Valk

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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.
Original languageEnglish
Pages (from-to)966-975
Number of pages10
JournalJournal of surgical oncology
Issue number6
Publication statusPublished - 1 Nov 2019


  • Zollinger-Ellison syndrome
  • multiple endocrine neoplasia type 1
  • neuroendocrine tumor
  • oncology

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