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

Figure 1

Surgical treatment algorithm for Zollinger-Ellison syndrome. ZES: Zollinger-Ellison syndrome; MEN1: Multiple endocrine neoplasia type 1; MEN1/ZES: Multiple endocrine neoplasia type 1-related Zollinger-Ellison syndrome; MEN1/HPT/ZES: Patients with primary hyperparathyroidism and Multiple endocrine neoplasia type 1-related Zollinger-Ellison syndrome; PTX: Parathyroidectomy.