TY - JOUR
T1 - Prognostic factors and survival in MEN1 patients with gastrinomas
T2 - Results from the DutchMEN study group (DMSG)
AU - van Beek, Dirk-Jan
AU - Nell, Sjoerd
AU - Pieterman, Carolina R. C.
AU - de Herder, Wouter W.
AU - van de Ven, Annenienke C.
AU - Dekkers, Olaf M.
AU - van der Horst-Schrivers, Anouk N.
AU - Drent, Madeleine L.
AU - Bisschop, Peter H.
AU - Havekes, Bas
AU - Rinkes, Inne H. M. Borel
AU - Vriens, Menno R.
AU - Valk, Gerlof D.
N1 - Funding Information:
This study was supported by an unrestricted grant from Ipsen Pharmaceutical. The funding source had no influence on the study question, design, data acquisition, statistical analysis, and interpretation of data.
Publisher Copyright:
© 2019 The Authors. Journal of Surgical Oncology published by Wiley Periodicals, Inc.
PY - 2019/11
Y1 - 2019/11
N2 - 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.
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.
KW - multiple endocrine neoplasia type 1
KW - neuroendocrine tumor
KW - oncology
KW - Zollinger-Ellison syndrome
KW - ENDOCRINE NEOPLASIA TYPE-1
KW - ZOLLINGER-ELLISON-SYNDROME
KW - PANCREATIC NEUROENDOCRINE TUMORS
KW - NATURAL-HISTORY
KW - GUIDELINES
KW - MANAGEMENT
KW - DIAGNOSIS
KW - SURGERY
U2 - 10.1002/jso.25667
DO - 10.1002/jso.25667
M3 - Article
C2 - 31401809
SN - 0022-4790
VL - 120
SP - 966
EP - 975
JO - Journal of Surgical Oncology
JF - Journal of Surgical Oncology
IS - 6
ER -