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目的:提出适合中国肝母细胞瘤(HB)患者的危险分层方法，探讨高危HB患者的新治疗方法。方法:2014年9月至2018年9月，中山大学肿瘤防治中心和中山大学附属第一医院收治的年龄≤18岁的100例初治HB患者，根据临床分期、甲胎蛋白水平、病理分型等因素，分为极低危、低危、中危和高危4个危险层级。极低危患者手术后不行化疗，随访观察；低危患者术后用C5V方案(顺铂＋氟尿嘧啶＋长春新碱)化疗共4个周期；中危患者术前和术后行C5VD方案(顺铂＋氟尿嘧啶＋长春新碱＋阿霉素)化疗，共6～8个周期；高危患者术前和术后行C5VD方案与IIV方案(异环磷酰胺＋伊立替康＋长春新碱)交替化疗，共8个周期。结果:100例患者分为极低危2例，低危10例，中危51例，高危37例。化疗前有可评价病灶者83例，化疗后部分缓解65例，疾病稳定10例，疾病进展8例，一线化疗客观有效率为78.3%。中位随访20个月，复发和进展30例，死亡27例。全组患者2年无进展生存率为69.2%，2年总生存率为72.0%。其中极低危、低危、中危、高危的2年无进展生存率分别为100%、88.9%、75.3%和43.2%, 2年总生存率分别为100%、100%、81.0%和44.8%。结论:新的HB危险分层方法简便可行。根据新危险分层方法分层后，对患者采取不同的治疗策略，极低危、低危和中危HB可获得较好的预后，高危HB患者的生存率仍有待提高，需要探讨新的治疗策略。“,”Objective:To provide the risk stratification method of hepatoblastoma (HB) suitable for implementation in China and explore the new treatment method for high-risk HB patients.Methods:A total of 100 cases of children and adolescents under 18 years old with newly diagnosed HB in Sun Yat-sen University Cancer Center and Sun Yat-sen University First Affiliated Hospital from September 2014 to September 2018 were included. According to the clinical stage, AFP level, pathological subtype and other factors, patients were stratified into four groups: extremely low-, low-, intermediate- and high-risk. The patients at very low risk were treated with surgery only and followed-up. The patients at very low risk were treated with C5V(Cisplatin+ 5-Fluroracil+ Vincristine) regimen for 4 courses. The patients at intermediate risk were treated with C5VD(Cisplatin+ 5-Fluroracil+ Vincristine+ Doxorubicin)regimen before and after surgery for 6-8 courses. The patients at high risk were treated with C5VD and IIV (ifoshamide+ irinotecan+ vincristine) alternately before and after surgery for 8 courses.Results:One hundred patients were stratified into extremely low-risk, low-risk, medium-risk and high-risk groups for 2, 10, 51 and 37 cases, respectively. Eighty three cases had evaluable lesions before chemotherapy. Among them, 65 patients achieved partial remission, stable disease and progressive disease were observed in 10, and 8 cases, respectively, with a response rate of 78.3%. During a median follow-up of 20 months, 30 patients experienced tumor relapse or progression, and 27 of them died. The 2-years progression-free survival (PFS) and overall survival (OS) rates were 69.2% and 72.0%, respectively. The 2-years PFS rates of patients with extremely low risk, low risk, medium risk and high risk were 100%, 88.9%, 75.3% and 43.2%, respectively. The 2-years OS rates were 100%, 100%, 81.0% and 44.8%, respectively.Conclusions:The novel HB risk classification is simple and feasible. With active comprehensive treatment, patients at extremely low-, low- and medium-risk have excellent outcomes. The survival rate of high-risk HB patients remains to be improved, and new treatment strategies need to be explored.