Liver Transplantation for Primary Hepatic Tumors in Children / 대한이식학회지

ABSTRACT

BACKGROUND: Primary liver tumors account for less than 2% of pediatric malignancies, and the best treatment is complete surgical excision. The aim of this study was to review the results of liver transplantation (LT) for primary hepatomas in children. METHODS: The medical records of patients who underwent LT for unresectable primary hepatoma between May 1996 and December 2009 were reviewed retrospectively. RESULTS: Seven of 130 patients (5.3%, MF=43) underwent LT for unresectable hepatoma. The median age at transplantation was 9 years (range, 6 months-14 years). Two patients were transplanted for hepatitis B virus-associated hepatocellular carcinoma (HCC), 2 for hepatoblastoma, 1 for hemangioendothelioma, 1 for angiosarcoma, and 1 for intrahepatic cholangiocarcinoma after a Kasai operation for biliary atresia. There was no post-LT treatment except in patients with HCC who were taking immunoglobulin prophylaxis against hepatitis B. Four patients (2 HCC, 1 hepatoblastoma, 1 hemangioendothelioma) are now alive and well after 7.8, 7.2, 7.7, 6.3 years of follow-up, respectively. Three patients died after transplantation; 1 for the recurrent cholangiocarcinoma in the transplanted liver 1 year after the transplantation and 1 who underwent LT for the recurrent hepatoblastoma for the primary non-function 10 days after the transplantation. One patient died of metastatic angiosarcoma (bone) 2.5 years after LT. CONCLUSIONS: LT can be tried for unresectable primary hepatoma in children and, although limited, the outcome was successful in patients with HCC, hepatoblastoma, or hemangioendothelioma. Careful patient selection, based on the pre-transplant histological diagnosis, seems to be related to better outcome.