Controversies in Surgery for Multiple Endocrine Neoplasia Type 1-Associated Zollinger–Ellison Syndrome

Abstract

Zollinger–Ellison syndrome (ZES) is a common manifestation of multiple endocrine neoplasia type 1 (MEN1). At least 90% of MEN1-ZES patients have multiple duodenal gastrinomas, making the duodenum the target organ of ZES. The indication and the timing of surgery in MEN1-ZES is controversial, since there is yet no parameter that indicates an aggressive course of disease and long-term survival is generally good. An imageable, most likely nonfunctioning pancreatic neuroendocrine neoplasm (pNEN) >1–2 cm seems to be a good surrogate parameter to indicate surgery in order to prevent distant metastatic pNEN disease, although some groups indicate surgery at the time of biochemical ZES evidence. The optimal surgical procedure is also controversial. Different strategies encomprise local excision via duodenotomy with or without distal pancreatic resection and regional lymphadenectomy to partial pancreaticoduodenectomy. At present, the timing and type of surgery for MEN1-ZES should be individualized according to patient’s characteristics and preference.