Hepatocellular carcinoma with fibrolamellar pattern in a patient with autoimmune cholangitis

A 75-year-old woman with a 15-year history of autoimmune cholangitis underw ent orthotopic liver transplantation because of progressive liver decompens ation. A clinically unsuspected hepatocellular carcinoma was found. A porti on of the tumor showed fibrolamellar differentiation. Hepatocellular carcin oma, 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 sett ing of autoimmune hepatitis. Autoimmune cholangitis is a relatively recentl y recognized form of autoimmune liver disease whose association with hepato cellular carcinoma has yet to be determined. Key Words: Autoimmune cholangi tis, primary biliary cirrhosis, hepatocellular carcinoma, fibrolamellar hep atocellular carcinoma.