Surgical management of Zollinger-Ellison syndrome: Classical considerations and current controversies

Abstract

Zollinger-Ellison syndrome (ZES) is characterized by gastric acid hypersecretion causing severe recurrent acid-related peptic disease. Excessive secretion of gastrin can now be effectively controlled with powerful proton pump inhibitors, but surgical management to control gastrinoma itself remains controversial. Based on a thorough literature review, we design a surgical algorithm for ZES and list some significant consensus findings and recommendations: (1) For sporadic ZES, surgery should be routinely undertaken as early as possible not only for patients with a precisely localized diagnosis but also for those with negative imaging findings. The surgical approach for sporadic ZES depends on the lesion location (including the duodenum, pancreas, lymph nodes, hepatobiliary tract, stomach, and some extremely rare sites such as the ovaries, heart, omentum, and jejunum). Intraoperative liver exploration and lymphadenectomy should be routinely performed; (2) For multiple endocrine neoplasia type 1-related ZES (MEN1/ZES), surgery should not be performed routinely except for lesions > 2 cm. An attempt to perform radical resection (pancreaticoduodenectomy followed by lymphadenectomy) can be made. The ameliorating effect of parathyroid surgery should be considered, and parathyroidectomy should be performed first before any abdominal surgery for ZES; and (3) For hepatic metastatic disease, hepatic resection should be routinely performed. Currently, liver transplantation is still considered an investigational therapeutic approach for ZES. Well-designed prospective studies are desperately needed to further verify and modify the current considerations.

Keywords: Zollinger-Ellison syndrome, Sporadic gastrinomas, Multiple endocrine neoplasia type 1, Hepatic metastatic disease, Surgical treatment

Core tip: Surgical approach to gastrinoma differs in sporadic Zollinger-Ellison syndrome (ZES) and multiple endocrine neoplasia type 1-related ZES (MEN1/ZES), and the role of surgery in patients with ZES is controversial, especially in those with MEN1. The current article will present classical considerations as well as the controversies that exist today with regard to the surgical management of ZES. We also make a surgical treatment algorithm for ZES based on a thorough literature review and the evidence provided by the current papers. To our best knowledge, this is the first review focusing on the surgical treatment of ZES and giving a detail as well as through discussion on this topic.