Non-functioning well-differentiated neuroendocrine tumor of the extrahepatic bile duct: an unusual suspect?

BACKGROUND

Neuroendocrine tumors (NETs) arising in the biliary tree are extremely rare, and 37 cases were identified in the English literature.

METHODS

A well-differentiated NET was found arising from the junction of the cystic and common hepatic ducts, in a 51-year-old male presenting with pedal edema and weight loss with abnormal liver enzymes and a normal serum bilirubin level. No mass was seen on radiological imaging and biopsy of the liver was suggestive of an early cholangiopathy. A bile leak complicating the liver biopsy led to an ERCP that demonstrated a filling defect suggestive of a mass in the common bile duct (CBD).

RESULTS

He underwent a successful excision of the tumor with a Roux-en-Y hepaticojejunostomy. The diagnosis of NET was made on histological and immunohistochemical analysis of the resected specimen. He remains well and disease free 22 months after surgery.

CONCLUSIONS

Recognition of biliary NET continues to be a challenge and an increased awareness of these tumors in rare sites will result in optimal management of these tumors.