Hepatocellular carcinoma in a long-term survivor of intrahepatic biliary duct hypoplasia.

We describe the development of hepatocellular carcinoma (HCC) in a 46-yr-old woman with intrahepatic biliary duct hypoplasia. Her underlying liver disease was quiescent for many years until a dramatic rise in serum bilirubin was seen. Orthotopic liver transplantation was performed, and a large tumor was found during surgery. A bile-producing HCC was identified, infiltrating about 50% of an otherwise noncirrhotic liver which had a paucity of intrahepatic ducts. The findings of HCC in this long-term survivor of intrahepatic biliary duct hypoplasia indicates HCC may occur in patients with long-standing disease.