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

• Published in: Journal of surgical oncology Vol. 120; no. 6; pp. 966 - 975
• Main Authors: Beek, Dirk‐Jan, Nell, Sjoerd, Pieterman, Carolina R.C., Herder, Wouter W., Ven, Annenienke C., Dekkers, Olaf M., Horst‐Schrivers, Anouk N., Drent, Madeleine L., Bisschop, Peter H., Havekes, Bas, Borel Rinkes, Inne H. M., Vriens, Menno R., Valk, Gerlof D.
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01.11.2019; John Wiley and Sons Inc
• Subjects: Cancer surgery; Cohort Studies; Female; Follow-Up Studies; Gastrinoma - metabolism; Gastrinoma - mortality; Gastrinoma - pathology; Gastrinoma - surgery; Humans; Intestinal Neoplasms - metabolism; Intestinal Neoplasms - mortality; Intestinal Neoplasms - pathology; Intestinal Neoplasms - surgery; Liver Neoplasms - metabolism; Liver Neoplasms - mortality; Liver Neoplasms - secondary; Liver Neoplasms - surgery; Male; Medical prognosis; Middle Aged; multiple endocrine neoplasia type 1; Netherlands; neuroendocrine tumor; Neuroendocrine tumors; Neuroendocrine Tumors - metabolism; Neuroendocrine Tumors - mortality; Neuroendocrine Tumors - pathology; Neuroendocrine Tumors - surgery; oncology; Pancreatic Neoplasms - metabolism; Pancreatic Neoplasms - mortality; Pancreatic Neoplasms - pathology; Pancreatic Neoplasms - surgery; Prognosis; Proto-Oncogene Proteins - metabolism; Stomach Neoplasms - metabolism; Stomach Neoplasms - mortality; Stomach Neoplasms - pathology; Stomach Neoplasms - surgery; Survival Rate; Zollinger‐Ellison syndrome; Netherlands; multiple endocrine neoplasia type 1; neuroendocrine tumor; oncology; Zollinger-Ellison syndrome
• ISSN: 0022-4790; 1096-9098
• DOI: 10.1002/jso.25667

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.