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.

Additional Metadata
Keywords multiple endocrine neoplasia type 1, neuroendocrine tumor, oncology, Zollinger-Ellison syndrome
Persistent URL dx.doi.org/10.1002/jso.25667, hdl.handle.net/1765/118670
Journal Journal of Surgical Oncology
Citation
van Beek, D.-J. (Dirk-Jan), Nell, S, Pieterman, C.R.C, de Herder, W.W, van de Ven, A.C, Dekkers, O.M, … Valk, G.D. (2019). Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG). Journal of Surgical Oncology. doi:10.1002/jso.25667