14例肝母细胞瘤患儿的临床疗效观察

目的：通过对儿童肝母细胞瘤患儿的临床治疗结果的回顾总结，对ICE化疗方案的有效性和安全性进行评估。方法：自2000年6月至2008年6月，14例初发患儿入选，男7例，女7例，中位年龄：1.33岁（范围0.25～8.25岁）。临床分期：Ⅰ期6例，Ⅱ期1例，Ⅲ期5例，Ⅳ期2例。诊断时血甲胎蛋白（AFP）水平显著升高13例，1例AFP正常。采用多科室协作模式进行治疗，其中一期手术8例，3例进行了二期手术。化疗方案采用ICE方案，14例患儿共接受了73个疗程化疗，其中术前化疗25个疗程。结果：14例患儿治疗后有效12例（85.7%）,其中完全缓解10例（71.4%），部分缓解2例，2例无效。随访至2008年7月31日，疾病处于长期完全缓解者9例（64.3%），中位随访时间为35个月（范围：16～96个月）。5年总生存率（OS）为：（70.71±12.37）%，5年无事件生存率（EFS）为：（64.29±12.81）%。1例患儿复发，2例失访。结论：ICE化疗方案联合手术治疗能有效并且安全地治疗儿童肝母细胞瘤，Ⅳ期患儿的治疗有待于进一步研究。［中国当代儿科杂志，2009，11（8）：659-662］

Clinical observation on the treatment outcome of 14 children with hepatoblastoma in a single medical center

Objective To evaluate the efficacy and safety of the ICE regimen(iphosphamide + carboplatin+etoposide)used in treating children with hepatoblastoma in the Shanghai Children's Medical Center.Methods From June 2000 to June 2008,14 children with newly diagnosed hepatoblastoma(7 males and 7 females)were enrolled.Their median age on diagnosis was 1.33 years(range:0.25-8.25 years).Six patients had stage I disease,1 had stage II,5 had stage III,and 2 had stage IV diseases.Thirteen children had markedly increased serum AFP level,and 1 had normal serum AFP level.Multidisciplinary co-operation treatment was performed.Eight patients had primary surgery while 3 patients had pre-operation chemotherapy before surgery.ICE chemotherapy regimen was used.Totally,73 courses of chemotherapy were administered for the 14 children and 25 out of the 73 courses were performed before operation.Results Twelve patients responded to the treatment(85.7%)and 2 failed.Ten patients(71.4%)achieved complete remission after treatment,and two had partial remission.By July 31st,2008,9 patients survived without any event,with a median follow-up duration of 35 months(range:16-96 months).The 5-year overall survival rate was 70.71±12.37%,and the 5-year event-free survival rate was 64.29±12.81%.One patient had disease relapse and two patients were lost to follow-up after they achieved partial remission.Conclusions The ICE regimen combined with operation is effective and safe in treating children with hepatoblastoma.