Biliary tree gastrinomas in multiple endocrine neoplasia type 1 syndrome

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

Figure 1

Computed tomography scan. A: The tumor (arrow) near the glissonian capsule of the right portal triad sheet; B: The lesion was positive at somatostatin receptor scintigraphy; C: Intraoperative picture: the tumor is close to the hepatic hilum, as indicated by the surgical forceps; D: Surgical resection of the V-VIII hepatic segments: the nodule is shown by the surgical forceps; E: Overview of the neuroendocrine tumor found inside the glissonian capsule of the right portal triad sheet; F: Tumor displaying distinct trabecular pattern; G: Massive gastrin production by the majority of neoplastic cells is shown by immunophenotyping.