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 - Borel Rinkes, Inne H M
AU - Vriens, Menno R
AU - Valk, Gerlof D
N1 - © 2019 The Authors. Journal of Surgical Oncology published by Wiley Periodicals, Inc.
PY - 2019/11/1
Y1 - 2019/11/1
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 - Zollinger-Ellison syndrome
KW - multiple endocrine neoplasia type 1
KW - neuroendocrine tumor
KW - oncology
KW - Prognosis
KW - Follow-Up Studies
KW - Intestinal Neoplasms/metabolism
KW - Gastrinoma/metabolism
KW - Humans
KW - Middle Aged
KW - Neuroendocrine Tumors/metabolism
KW - Proto-Oncogene Proteins/metabolism
KW - Male
KW - Survival Rate
KW - Pancreatic Neoplasms/metabolism
KW - Stomach Neoplasms/metabolism
KW - Netherlands
KW - Female
KW - Cohort Studies
KW - Liver Neoplasms/metabolism
UR - http://www.scopus.com/inward/record.url?scp=85070355954&partnerID=8YFLogxK
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 -