A Clinical Study on Hepatoblasoma in Children / 대한소아혈액종양학회지

ABSTRACT

PURPOSE: Hepatoblastoma comprises over two-thirds of the malignant tumors of the liver in childhood. Although complete resection is the cornerstone of successful management, combination chemotherapy has had a major impact in improving survival. The purpose of this report is to review the clinical finding, therapeutic response, and long- term result of combined modality in 10 children who diagnosed hepatoblastoma. METHODS: From September 1993 to May 1998, the initial diagnosis of hepatoblastoma was made in 10 children at Severance hospital. We analysed medical record retrospectively for clinical finding, therapeutic modality, and survival. RESULTS: 1) The 10 children ranged in age from 3 months to 51 months of age at diagnosis (median age 21 months of age). 2) There were 9 boys and 1 girl 3) The children presented with either abdominal distension or a mass. Of 10 patients, 6 patients had thrombocytosis more than 500,000/muL and 7 patients had elevated alpha-fetoprotein (AFP) more than 30,000 IU/mL. 4) Pathology results were predominantly of epithelial histology except one case that had elements of mixed. 5) 7 children had right lobe involvement and one child had tumor in both lobe. 6) Surgery was undertaken at diagnosis in 6 patients and preoperative chemotherapy was taken in 4 patients. Preoperative chemotherapy significantly reduced the extent of resection to be necessary for complete resection in 3 patients. 7) 8 of 10 children (80%) are alive (4 to 51 months posttreatment) with no evidence of disease with median follow-up of 39 months. 2 children are lost to follow-up. Conclusions: Excellent cure rates has been achived with complete resection followed by multiagent chemotherapy for hepatoblastoma. Future studies are directed toward the identification of poor risk patients with protocols designed to improve survival for children with advanced disease.