Zollinger-Ellison Syndrome due to Primary Gastrinoma of The Extrahepatic Biliary Tree: Three Case Reports and Review of Literature

ObjectiveTo report 3 cases of primary neuroendocrine tumors (PNT) of the extrahepatic biliary tree (EHBT) in patients with Zollinger-Ellison syndrome (ZES), 2 of whom had multiple endocrine neoplasia type 1 (MEN 1).MethodsThree new cases of gastrin-producing tumors of the EHBT are presented, and the pertinent literature relating to PNT of the EHBT is reviewed.ResultsEighty-one previous cases of PNT of the EHBT have been reported in the world literature, 7 of which were hormonally active and associated with peptic ulcer disease, diarrhea, or ZES. Three additional patients presented to us with ZES due to PNT of the EHBT. One patient with MEN 1 was treated with a Whipple procedure for a common bile duct gastrinoma. A second patient underwent left hepatectomy with resection of the confluence of the right and left hepatic ducts for a primary left hepatic duct gastrinoma. The second patient with MEN 1 underwent resection of a gastrinoma at the junction of the cystic duct and the common bile duct.ConclusionAlthough PNT of the EHBT are uncommon, the association with ZES is even more so. Often, nonfunctioning tumors are diagnosed late in the course of the disease from symptoms related to biliary obstruction. Patients with ZES may be diagnosed earlier because of symptoms resulting from gastrin excess. Surgical resection is the only chance for cure and is often helpful in the palliation of symptoms. (Endocr Pract. 2009;15:737-749)