Brief Article
Copyright ©2013 Baishideng Publishing Group Co., Limited. All rights reserved.
World J Gastroenterol. Dec 7, 2013; 19(45): 8312-8320
Published online Dec 7, 2013. doi: 10.3748/wjg.v19.i45.8312
Biliary tree gastrinomas in multiple endocrine neoplasia type 1 syndrome
Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi
Francesco Tonelli, Francesco Giudici, Gabriella Nesi, Giacomo Batignani, Maria Luisa Brandi, Surgical Unit, Department of Surgery and Translational Medicine, University of Florence, 50014 Florence, Italy
Author contributions: All authors contributed equally to this work; Tonelli F, Giudici F and Batignani G collected the data; Tonelli F, Giudici F, Nesi G, Batignani G and Brandi ML analyzed data and wrote the manuscript; Brandi ML supervised the study.
Correspondence to: Francesco Tonelli, MD, Surgery Unit, Department of Clinical Physiopathology, University of Florence, Largo Brambilla 3, 50014 Florence, Italy. francesco.tonelli@unifi.it
Telephone: +39-55-7947449 Fax: +39-55-7947449
Received: April 29, 2013
Revised: June 29, 2013
Accepted: July 4, 2013
Published online: December 7, 2013
Processing time: 232 Days and 11.5 Hours
Abstract

AIM: To describe our patients affected with ectopic biliary tree gastrinoma and review the literature on this topic.

METHODS: Between January 1992 and June 2012, 28 patients affected by duodenopancreatic endocrine tumors in multiple endocrine neoplasia type 1 (MEN1) syndrome underwent surgery at our institution. This retrospective review article analyzes our experience regarding seventeen of these patients subjected to duodenopancreatic surgery for Zollinger-Ellison syndrome (ZES). Surgical treatment consisted of duodenopancreatectomy (DP) or total pancreatectomy (TP). Regional lymphadenectomy was always performed. Any hepatic tumoral lesions found were removed during surgery. In MEN1 patients, removal of duodenal lesions can sometimes lead to persistence or recurrence of hypergastrinemia. One possible explanation for this unfavorable outcome could be unrecognized ectopic localization of gastrin-secreting tumors. This study described three cases among the seventeen patients who were found to have an ectopic gastrinoma located in the biliary tree.

RESULTS: Seventeen MEN1 patients affected with ZES were analyzed. The mean age was 40 years. Fifteen patients underwent DP and two TP. On histopathological examination, duodeno pancreatic endocrine tumors were found in all 17 patients. Eighty-one gastrinomas were detected in the first three portions of the duodenum. Only one gastrinoma was found in the pancreas. The mean number of gastrinomas per patient was 5 (range 1-16). Malignancy was established in 12 patients (70.5%) after lymph node, liver and omental metastases were found. Three patients exhibited biliary tree gastrinomas as well as duodenal gastrinoma(s). In two cases, the ectopic gastrinoma was removed at the same time as pancreatic surgery, while in the third case, the biliary tree gastrinoma was resected one year after DP because of recurrence of ZES.

CONCLUSION: These findings suggest the importance of checking for the presence of ectopic gastrinomas in the biliary tree in MEN1 patients undergoing ZES surgery.

Keywords: Gastrinoma; Multiple endocrine neoplasia type 1; Zollinger-Ellison Syndrome; Ectopic gastrinoma; Biliary tree; Duodenopancreatectomy

Core tip: Enteropancreatic endocrine neoplasms affect up to 90% of multiple endocrine neoplasia type1 (MEN1) patients. Gastrinomas are the most common functional enteropancreatic neuroendocrine tumors and were thought to be located almost exclusively in the duodenum in MEN1 patients. This study describes our experience regarding ectopic biliary tree gastrinomas in MEN1 syndrome. Our data doubles the number of cases reported in literature on this topic. Furthermore, the present study brings to light important issues that could help to establish the best biochemical and oncological cure for such cases, of which clinicians should be aware to improve the management of MEN1 patients.