Prognostic factors and survival in MEN1 patients with gastrinomas: Results from the DutchMEN study group (DMSG). Issue 6 (10th August 2019)

• Record Type: Journal Article
• Authors: van Beek, Dirk‐Jan; Nell, Sjoerd; Pieterman, Carolina R.C.; de Herder, Wouter W.; van de Ven, Annenienke C.; Dekkers, Olaf M.; van der Horst‐Schrivers, Anouk N.; Drent, Madeleine L.; Bisschop, Peter H.; Havekes, Bas; Borel Rinkes, Inne H. M.; Vriens, Menno R.; Valk, Gerlof D.
• Is Part Of: Journal of surgical oncology. Volume 120:Issue 6(2019)
• Page Start: 966
• Page End: 975
• Publication Date: 2019-08-10
• Subjects: multiple endocrine neoplasia type 1 -- neuroendocrine tumor -- oncology -- Zollinger‐Ellison syndrome; Cancer -- Surgery -- Periodicals; Neoplasms -- Periodicals; 616
• DOI: 10.1002/jso.25667
• Languages: English
• ISSNs: 0022-4790

Abstract

Abstract: 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.