Improved long-term survival with preoperative chemotherapy for hepatoblastoma

In 1986, The Hospital for Sick Children (Toronto, Canada) began to use a standard preoperative chemotherapeutic regimen for patients who had unresectable hepatoblastoma. In 1988, we extended this protocol to all children who had hepatoblastoma. Of 25 children who presented with hepatoblastoma, 22 were eligible for protocol therapy. After percutaneous biopsy, cycles of cisplatin (20 mg/m2/d for 5 days) and Adriamycin (25 mg/m2/d for 3 days) were administered every 3 weeks by continuous intravenous infusion. A CAT scan was performed after the third cycle. Surgery was undertaken if response indicated that complete resection was possible. If not, a further one to three cycles were given until response was adequate. Postoperatively, therapy was continued for a total of six cycles. Twenty of twenty-two (91%) tumors responded to chemotherapy. Over half required only three cycles. Twenty hepatic resections (6 segmentectomies, 10 lobectomies, 4 trisegmentectomies) were performed. Preoperative therapy significantly reduced the extent of resection calculated to be necessary for complete excision at an initial diagnosis of the primary tumor in all but one. In the two children with inadequate response, total hepatectomy and transplant was necessary for complete resection. No deaths or operative delays were attributed to chemotherapy toxicity. Nineteen of 22 children (87%) are alive with no evidence of disease, including both transplant patients. One death was caused by intraoperative bleeding and the other two were caused by metastatic lung disease at 22 and 26 months, respectively. Twelve children, eight with tumors that would have been unresectable before effective chemotherapy, have had follow-up for more than 5 years. This protocol of preoperative chemotherapy appears to be safe and effective for most children who have hepatoblastoma.