Bile duct carcinoma: a late complication of congenital hepatic fibrosis. Case report and review of literature.

Bile duct carcinoma is reported in a 50-year old man as a rare late presentation of congenital hepatic fibrosis (CHF). A survey of the world literature suggests that carcinoma occurs with increased frequency in congenital biliary dilatation. It appears to be a particular risk in patients with prolonged survival but is rare in CHF, where portal hypertension and renal insufficiency make the long-term prognosis poor. In this patient the absence of both gastroesophageal varices and renal disease may have permitted longevity with development of carcinoma. The finding of carcinoma arising in the bile ducts distal to the basic lesion of CHF suggests that active carcinogens may be present in the bile in CHF. The tumor in this patient was unusual in producing mucinous biliary obstruction and terminal hypercalcemia.