To evaluate clinical and laboratory findings of these patients and the efficacy of
liver transplantation in children with
hepatocellular carcinoma (HCC) and
hepatoblastoma (HB) associated with
tyrosinemia. Among 113 children with liver
tumors diagnosed between 1972 and 2004 five patients had HCC or HB associated with
tyrosinemia. The age at diagnosis of the HCC or HB ranged from 9.5 to 17 yr and male:female ratio was 1:4. During regular
clinic visits for
tyrosinemia, elevated
alpha-fetoprotein (AFP) was detected in all patients. AFP levels ranged between 13.7 and 29 340 IU/mL. Radiological studies including ultrasound, computed tomography and magnetic resonance imaging showed heterogeneous parenchyma and nodules in the liver. The patients did not have any metastatic disease. The time from diagnosis of
tyrosinemia to HCC or HB ranged from 9.25 to 15.25 yr. Histopathologically, four patients have been diagnosed as HCC and one patient had HB. All patients were given
chemotherapy including
cisplatin and
adriamycin. In three patients, living-related
liver transplantation was performed. They had no treatment after
transplantation. All of them are disease free. One patient was treated with
chemotherapy and right
hepatectomy. She had no suitable donor for living-related
liver transplantation. Three months after completing
chemotherapy, she had recurrent
tumor in the left lobe of the liver and she died with progressive disease. The last patient whose parents were not suitable as donors for living-related
liver transplantation is waiting for a deceased donor graft. All patients had limited disease to liver due to close clinical and radiological follow up for
tyrosinemia. In these patients
liver transplantation is curative both for liver
tumor and
tyrosinemia.