Hepatocarcinoma in association with the Alagille syndrome

An unusual image pattern of hepatocyte function in a 31-year-old man with the Alagille syndrome is presented. The Alagille syndrome is comprised of a constellation of findings, including characteristic facies, vertebral anomalies, retarded physical and sexual development, ocular posterior embryotoxon, peripheral pulmonary artery stenosis and intrahepatic ductular hypoplasia. Radiographic investigation of this patient with eventual biopsy also revealed the presence of hepatocellular carcinoma. This is an extremely rare association of these conditions and represents the third reported case.