Hepatocellular carcinoma with fibrolamellar pattern in a patient with autoimmune cholangitis.

A 75-year-old woman with a 15-year history of autoimmune cholangitis underwent orthotopic liver transplantation because of progressive liver decompensation. A clinically unsuspected hepatocellular carcinoma was found. A portion of the tumor showed fibrolamellar differentiation. Hepatocellular carcinoma, either with the usual pattern or with a fibrolamellar pattern, is rare in the setting of primary biliary cirrhosis, but has been seen in the setting of autoimmune hepatitis. Autoimmune cholangitis is a relatively recently recognized form of autoimmune liver disease whose association with hepatocellular carcinoma has yet to be determined.