Carcinoid tumors of the extrahepatic bile duct

BACKGROUND Carcinoid tumors of the extrahepatic bile duct are rare and account for 0.2–2% of all gastrointestinal carcinoids. Similar to other tumors of the bile duct, these lesions are difficult to diagnose preoperatively and nearly impossible to distinguish from cholangiocarcinoma. METHODS The authors retrospectively analyzed all reported cases of carcinoid tumor of the bile duct and report on two additional cases. RESULTS Thirty cases of carcinoid tumor of the bile duct have been reported. Among this group were 20 women and 9 men (female‐to‐male ratio, 2.2:1) with an overall mean age of 47 years (range, 19–79 years). The most common anatomic sites for extrahepatic carcinoid tumors were the common bile duct (58%), perihilar region (28%), cystic duct (11%), and common hepatic duct (3%). Jaundice was the most common presenting complaint (55%). Sixty‐nine percent of patients with extrahepatic biliary carcinoids had disease confined to the bile duct, whereas 31% had evidence of distant metastases. All patients who presented with localized disease remain disease free with a mean follow‐up of 32 months (range, 3 months to 20 years). CONCLUSIONS Carcinoid tumor of the bile duct is a rare form of malignant biliary obstruction. Unlike cholangiocarcinoma, biliary carcinoids occur more commonly in younger patients and in women. Aggressive local invasion by the primary tumor is rare, and metastases occur in less than one‐third of patients. All patients who underwent a curative surgical resection were alive and disease free at time of published report, implying a more favorable prognosis. Aggressive surgical resection is recommended. Cancer 1999;86:1959–65. © 1999 American Cancer Society.