Hepatoblastoma presenting with lung metastases: treatment results of the first cooperative, prospective study of the International Society of Paediatric Oncology on childhood liver tumors.

摘要: BACKGROUND The prognosis of children who are affected by hepatoblastoma (HB) that presents with lung metastases has always been considered very poor. In light the overall improvement in survival HB patients since introduction cisplatin (CDDP) therapeutic armament this tumor, question raised whether metastatic also would benefit from drug. The purpose current study was to address issue analyzing treatment outcome those presenting entered into first on childhood liver tumors conducted International Society Paediatric Oncology (SIOPEL 1). METHODS SIOPEL 1 a prospective, international, multicentric, single-arm based preoperative chemotherapy open patient registration January 1990 February 1994. After undergoing biopsy, received four courses CDDP (80 mg/m2 24-hour, continuous infusion) Day followed doxorubicin (60 48-hour, Days 2 and 3 (PLADO). Surgery performed after PLADO two more courses. Untreated age < 16 years biopsy-proven were eligible for study. Metastatic spread assessed chest X-ray and, where available, computed tomography scan. RESULTS Thirty-one 154 trial presented metastases. Eight presently alive no evidence disease (NED) being treated protocol therapy only (median follow-up, 60 months); nine NED having failed rescued alternative therapies 80 months). 5-year event free rates these 57% (95% confidence interval, 39–75%) 28% 12–44%), respectively. Persistent main reason failure (17 23 patients; 74%). CONCLUSIONS The SIOPEL strategy seems cure 25% present However, further use thoracotomies still can save significant numbers children. Cancer 2000;89:1845–53. © 2000 American Society.