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World J Gastroenterol. Aug 28, 2019; 25(32): 4673-4681
Published online Aug 28, 2019. doi: 10.3748/wjg.v25.i32.4673
Surgical management of Zollinger-Ellison syndrome: Classical considerations and current controversies
Qian-Qian Shao, Bang-Bo Zhao, Liang-Bo Dong, Hong-Tao Cao, Wei-Bin Wang
Qian-Qian Shao, Bang-Bo Zhao, Liang-Bo Dong, Hong-Tao Cao, Wei-Bin Wang, Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences/Peking Union Medical College, Beijing 100730, China
Author contributions: Shao QQ, Dong LB and Zhao BB performed the literature review; Shao QQ and Cao HT wrote the manuscript; Wang WB was involved in reviewing and editing the manuscript.
Supported by the National Natural Science Foundation, No. 81773215; and the General Financial Grant from the China Postdoctoral Science Foundation, No. 2017M610813.
Conflict-of-interest statement: All authors state that they have no competing interests.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Wei-Bin Wang, MD, Professor, Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences/Peking Union Medical College, Dongcheng District, Beijing 100730, China.
Telephone: +86-10- 69152600 Fax: +86-10-69152600
Received: March 2, 2019
Peer-review started: March 2, 2019
First decision: April 4, 2019
Revised: April 29, 2019
Accepted: May 3, 2019
Article in press: May 3, 2019
Published online: August 28, 2019

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.