Bulky gastrinoma of the common bile duct: Unusual localization of extrapancreatic gastrinoma - case report

Abstract

Pancreatic endocrine tumors may present as isolated tumors or as part of a genetic syndrome such as multiple endocrine neoplasia type I (MEN-I).These tumors are characterized by the secretion of one or more peptide hormones resulting in a constellation of symptoms attributed to hormone ex-

Incidence and Prognosis of Primary Gastrinomas in the Hepatobiliary Tract

“…This distribution is at odds with the frequency of possible hepatic or biliary tract gastrinomas causing ZES reported in the literature. Previous reviews of literature on hepatobiliary NETs report that 20 to 25 patients with sporadic ZES and proposed hepatic gastrinomas have been described compared with only 5 patients with sporadic ZES and possible biliary tract gastrinomas, [16][17][18]23,36 which suggests that hepatic gastrinomas are more frequent than biliary tract gastrinomas. Coupled with the fact that biliary tract gastrinomas can be easily missed because they can occur within the bile duct or can be attached to the duct and mistaken for possible lymph node metastases, our data raise the possibility that gastrinomas at this site occur more frequently than previously reported.…”

“…37,45,64 Furthermore, duodenal gastrinomas can be small (1-5 mm) and easily missed 42,43,59 ; thus, another site could be thought to be a primary site. Primary hepatobiliary gastrinomas have been reported in a small number of case reports (<3 cases/report) in patients with sporadic ZES 14,15,[17][18][19][20][21][23][24][25][26][27][28][29][30][31][32][33][34][35][36][37][38][39] and with MEN1 with ZES. 16,22 However, for the aforementioned reasons, their existence is questioned and their frequency and natural history, including presentation, resectability, possible cure, and pattern of recurrence, are unknown.…”

Neuroendocrine Tumors of the Hepatobiliary Tract

“…Bile duct gastrinomas can grow to large size. One patient had a solitary, well-encapsulated mass of 8 cm diameter in the common bile duct (Tarçin et al 2011). Among three patients reported by Price et al (2009), two had MEN1 syndrome.…”

“…The majority of the rare hepatobiliary gastrinomas are observed within the liver substance (see above), but few tumors were detected in the walls of the extrahepatic bile ducts (Mandujano-Vera et al 1995;Hao et al 1996;Martignoni et al 1999;Price et al 2009;Tarçin et al 2011). As with intrahepatic gastrinomas, these neoplasms may cause Zollinger-Ellison syndrome.…”

Zollinger–Ellison syndrome

“…This change is due to the fact that duodenal gastrinomas are frequently small(<1-cm), not seen on imaging and thus were easily missed in the early studies, and are still missed at surgery, if a routine duodenotomy isn’t performed[14,38,39,42,43]. Primary gastrinomas are uncommonly located in other intra-abdominal locations including:lymph nodes(controversial), stomach, mesentery, renal capsule, splenic hilum, omentum, ovary and in the liver/biliary tract[41,44,45,46●,47–49]. Rarely (<0.3%) primary gastrinomas may occur in extra-abdominal locations such as the heart and lung[41].…”