Cholangiocarcinoma arising in congenital hepatic fibrosis. A case report.

Congenital hepatic fibrosis (CHF) is a morphologic entity characterized by (1) abnormal fibrous septation, (2) preservation of the basic lobular architecture, and (3) hyperplasia and dilatation of bile ductules. Congenital hepatic fibrosis may present as a cause of portal hypertension in adolescents, in young adults, and, rarely, in older adults. It is this form of the disorder that has been emphasized in the literature. However, CHF may also be seen in association with the infantile polycystic kidney syndrome. In the latter, the hepatic disorder is usually clinically silent. Cholangiocarcinoma is a rare complication of comgenital hepatic fibrosis, with only two previously documented cases reported, to our knowledge. We report a third case and suggest that CHF may represent a dysplastic hamartoma that, with prolonged survival, may eventuate in the development of intrahepatic biliary neoplasia.