Multifocal hepatocellular carcinoma and precancerous lesions in a patient with autoimmune hepatitis-related cirrhosis.

A 44-year-old woman with a 29-year history of autoimmune hepatitis (AIH) received a living donor liver transplant for multifocal hepatocellular carcinoma (HCC) and cirrhosis in 2007. Her initial laboratory workup at our institution in 1996 revealed a positive antismooth muscle antibody with a titer of 1:640. Serum electrophoresis showed a monoclonal gamma globulin spike with elevated IgG, IgA, and IgM. The patient was negative for hepatitis B and hepatitis C (HCV) by serology and serum polymerase chain reaction. She was treated with corticosteroids and azathioprine, but her disease progressed. In 1997, a liver needle biopsy revealed cirrhosis and a focus of small cell change. In 2004, a 2-cm exophytic mass was detected on magnetic resonance imaging. Follow-up imaging in 2005 and 2006 showed growth of the exophytic mass and development of new tumors. The exophytic mass was treated with ethanol ablation and she received a transplant. Examination of the explant revealed multiple high-grade dysplastic nodules and four moderately differentiated HCCs, one of which is arising in a high-grade dysplastic nodule. We believe this to be the first case in the English literature documenting the presence of preneoplastic lesions in an HCV-negative patient with AIH who developed HCC.