IMPROVED OUTCOME FOR CHILDREN WITH HEPATOBLASTOMA

Between 1981 and 1993, 41 children were treated for hepatoblastoma. Cl inical, radiological and pathological data were reviewed retrospective ly, focusing on surgical aspects of treatment and outcome. Fourteen ch ildren underwent primary resection of the hepatic tumour. One infant w ith severe congenital anomalies received only palliative treatment. Of 26 with irresectable disease, pulsed cytotoxic chemotherapy (cisplati n and doxorubicin) enabled subsequent surgical excision in 22 and one child with persistent extensive intrahepatic disease was successfully treated by liver transplantation. Thus, with a policy of selective pre operative chemotherapy, 90 per cent of hepatoblastomas were resectable . There were no perioperative deaths from haemorrhage but one child di ed from an intraoperative tumour embolus. A total of 28 survivors, 27 of whom are disease-free, were followed for a median of 5 years. The c umulative probability of survival in patients treated with intent to c ure was 67 per cent. Analysis of survival data suggested a favourable outcome for those with a pure fetal histological tumour subtype. These results demonstrate significant progress in the treatment of hepatobl astoma.