An 11 year old girl, diagnosed as tyrosinosis at the age of 8, developed right upper quadrant pain. Liver scintigram showed space occupying lesion in the right lobe. The serum alpha-fetoprotein was also elevated. At laparotomy the liver showed cirrhotic and right hepatic lobectomy was carried out. Histopathologically tumor was adult type hepatocellular carcinoma and liver tissue showed macronodular cirrhosis. The serum alpha-fetoprotein level decreased rapidly following the operation. No adjuvant chemotherapy was performed because of impaired hepatic function. Four months later, alpha-fetoprotein level began to increase with signs of relapse and intra-arterial chemotherapy was attempted. However, its side effects made it impossible to continue the chemotherapy. The patient died of progressive disease 9 months after the operation. In Japan 19 cases of tyrosinosis have been reported but hepatomas were associated only in 2 cases of them. In these 2 cases the tumor was found at autopsy. The present case is the first one operated in Japan. Chronic form of tyrosinosis is always associated with liver cirrhosis and is often complicated with hepatoma. Although hepatocellular carcinoma in infancy and childhood is rarely associated with cirrhosis, patient with chronic form of tyrosinosis is the "high-risk" patient against hepatoma. The serum alpha-fetoprotein mesurement and other radiological diagnostic procedures are helpful to detect hepatoma in these patients.
