ABSTRACT
Primary endocrine neoplasms of intra- and extrahepatic biliary ducts are very rare. We describe the first case of a primary endocrine tumor of the common bile duct producing gastrin. A 53-year-old woman had a 3-year history of recurrent duodenal and gastric ulcers as well as obstructive jaundice. A small neoplasm was found in the lower third of the common bile duct, which showed diffuse gastrin production and focal synthesis of serotonin and pancreatic polypeptide by immunohistochemistry and electron microscopy. Although serum gastrin was within normal levels (90 ng/ml), symptoms of peptic acid disease could have been related to hypergastrinemia, since gastric and duodenal ulcers healed after surgical removal of the tumor. She has remained asymptomatic for 8 months.