Analysis of treatment efficiency of carboplatin and etoposide in combination with radical surgery in advanced and recurrent childhood hepatoblastoma:A report of the German Cooperative Pediatric Liver Tumor Study HB 89 and HE 94

Background: Hepatoblastoma (HB) is the most common liver tumor of childhood , and comprises approximately 1% of all pediatric malignancies. Although re cent data from multicenter trials of GPOH, SIOP, CCG and FOG indicate a rem arkable improvement of therapy results, the prognosis of advanced or recurr ent HB is still not satisfying. Patients and Methods: During 1989 and 1997, the German Cooperative Pediatri c Liver Tumor Studies HB 89 and HB 94 registered 141 patients with HB, who were treated according to the study protocols. These patients received stan dard chemotherapy with ifosfamide, cisplatin and doxorubicin (IPA) pre-oper atively and/or post-operatively. Fourteen children with recurrent or advanc ed HB were additionally treated with carboplatin and etoposide (CARBO/VP 16 ), the reason being observations of drug resistance in children with HB aft er four or more courses of IPA-therapy in the HB 89 study. The clinical data and course of these patients were evaluated to investigat e the efficiency of CARBO/VP 16 chemotherapy and for analyzing the role of surgery. Results: Mean follow-up for survivors was 4.3 years (range 13 months-8 year s). Tumor resection was attempted in 13 children but, in only 3 cases, was a complete tumor resection achieved in one operation. There was no perioper ative death, and 7 of the patients (50%) are in remission. Two patients und erwent adjuvant chemotherapy with CARBO/VP 16 for advanced HB at first oper ation: all are alive and well. Five patients with local relapse and/or dist ant metastases responded partially to CARBO/VP 16 therapy, and a complete r emission was achieved in one patient. In five patients, progressive disease was observed during therapy with CARBO/VP 16. One patient, stable while on chemotherapy, had a successful resection. Acute toxicity of chemotherapy w as observed in 7 patients (50%).