Abstract
AimTo assess postoperative complications and control of hormone secretions following pancreatoduodenectomy (PD) performed on multiple endocrine neoplasia type 1 (MEN1) patients with duodenopancreatic neuroendocrine tumors (DP-NETs).BackgroundThe use of PD to treat MEN1 remains controversial, and evaluating the right place of PD in MEN1 disease makes sense.MethodsThirty-one MEN1 patients from the Groupe d’étude des Tumeurs Endocrines MEN1 cohort who underwent PD for DP-NETs between 1971 and 2013 were included. Early and late postoperative complications, secretory control and overall survival were analyzed.ResultsIndication for surgery was: Zollinger–Ellison syndrome (n = 18; 58%), nonfunctioning tumor (n = 9; 29%), insulinoma (n = 2; 7%), VIPoma (n = 1; 3%) and glucagonoma (n = 1; 3%). Mean follow-up was 141 months (range 0–433). Pancreatic fistulas occurred in 5 patients (16.1%), distant metastases in 6 (mean onset of 43 months; range 13–110 months), postoperative diabetes mellitus in 7 (22%), and pancreatic exocrine insufficiency in 6 (19%). Five-year overall survival was 93.3% [CI 75.8–98.3] and ten-year overall survival was 89.1% [CI 69.6–96.4]. After a mean follow-up of 151 months (range 0–433), the biochemical cure rate for MEN-1 related gastrinomas was 61%.ConclusionIn MEN1 patients, pancreatoduodenectomy can be used to control hormone secretions (gastrin, glucagon, VIP) and to remove large NETs. PD was found to control gastrin secretions in about 60% of cases.
Highlights
Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant hereditary syndrome with a prevalence of 2/100,000 individuals
Pancreatic fistulas occurred in 5 patients (16.1%), distant metastases in 6, postoperative diabetes mellitus in 7 (22%), and pancreatic exocrine insufficiency in 6 (19%)
Zollinger–Ellison syndrome criteria were the presence of continuous specific clinical symptoms associated with ZES features found on endoscopy, an inability to discontinue high-dose proton pump inhibitors, and at least 2 out of the 4 National Institute of Health (NIH) criteria and a histological confirmation of gastrinoma [15, 16]
Summary
Multiple endocrine neoplasia type 1 (MEN1) is an autosomal dominant hereditary syndrome with a prevalence of 2/100,000 individuals. The most common MEN1 lesions are (in order of frequency) primary hyperparathyroidism, neuroendocrine duodenopancreatic tumors (DP-NETs) and pituitary tumors, neuroendocrine thymic tumors, bronchic tumors, and adrenal tumors. Surgery (pancreatoduodenectomy—PD) is recommended for large, non-functioning tumors ([ 2 cm in diameter) located on the head of the pancreas because of the risk of malignant spread [7, 8]. PD may be indicated in order to control the secretion of glucagonomas, Vipomas and insulinomas. Several authors have concluded that PD is the best option for patients with Zollinger–Ellison syndrome (ZES) because gastrinomas tend to be numerous and located in the duodenum [9,10,11]. Proton Pump Inhibitors (PPIs) can be an efficient, surgery-free means of controlling acid secretion, but surgery is more likely to prevent metastatic spread
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Free) 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call
