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目的 总结新生儿肝母细胞瘤的临床表现与治疗结果,提高对新生儿病例的认识,探讨更有效的治疗方案.方法 回顾性分析2007年至2013年在本院诊断及治疗的初诊年龄小于60 d的8例新生儿肝母细胞瘤病例,其中男6例,女2例,初诊年龄2～50d,平均(24.75±17.62)d,出生体重2.6～4.0kg,平均(3.42±0.47)kg.有7例肿瘤来源于右叶,1例来源于左叶.按照PRETEXT分期标准,Ⅰ期与Ⅱ期各1例,Ⅲ期6例.按照病理类型均为上皮型肝母细胞瘤,其中纯胎儿型1例,胚胎与胎儿混合型7例.Ⅰ期与Ⅱ期病例均行一期肿瘤根治性切除,6例Ⅲ期病例均经开腹肝脏肿瘤活检,明确病理类型后予辅助化疗,其中5例行延期肿瘤切除术.术前平均(4.17±1.16)个疗程,中位时间4疗程.肿瘤切除术后首次化疗时间平均为(22.00±9.42)d,中位时间23.5 d.术后化疗平均(5.00±2.52)个疗程,中位时间4.5疗程.结果 7例肿瘤切除术中有1例出现右肝管损伤,行肝管-空肠Roux-en-Y吻合术修复,其余6例手术无术后并发症.有2例(2/7,28.6％)术后复发,其余5例现均无瘤生存.中位随访时间29.5个月.Kaplan-Meier曲线计算后显示2年、5年的总体生存率分别为75％、60％.结论 正常足月新生儿肝母细胞瘤经过规范化治疗后仍具有良好的预后.由于新生儿肝母细胞瘤非常罕见,需要多中心、多学科联合研究.“,”Objective To summarize the clinical manifestations and outcomes of neonatal hepatoblastoma for its improved recognization and more effective treatment.Methods For this retrospective study,8 patients with neonatal hepotoblastoma were admitted from 2007 to 2014.Only neonates and infants aged under 2 months with adequate clinicopathologic data were accepted for review.There were 6 boys and 2 girls with a mean diagnostic age of 24.75 ± 17.62 (2-50) days.The mean birth weight at diagnosis was 3.42 ± 0.47 (2.6-4.0) kg.Seven tumors were derived from right hepatic lobe while another from left hepatic lobe.According to the criteria of PRETEXT,the stages were Ⅰ (n =1),Ⅱ (n =1) and Ⅲ (n =6).According to the histology classification,all tumors were of epithelial type.One patient was of pure fetal histology subtype while the remainder mixed fetal & embryonal subtype.Patients with stages Ⅰ and Ⅱ underwent primary operation while those with stage Ⅲ open biopsy and neoadjuvant chemotherapy.Five patients had delayed operation.The mean interval of preoperative chemotherapy was 4.17 ± 1.16 cycles,median 4 cycles.The mean time of initiating postoperative chemotherapy was 22.00 ± 9.42 days,median 23.5 days.The mean interval of postoperative chemotherapy was 5.00 ± 2.52 cycles,median 4.5cycles.Results Right hepatic duct was injured and subsequently repaired with cholangio-jejunostomy in 1 case.There were no other complications.Two tumors recurred postoperatively.Another 5 patients survived tumor-free.The median period of follow-up was 29.5 months.The 2,5-year overall survivals were 75％ and 60％ respectively.Conclusions Normal term newborns with hepatoblastoma have a good prognosis after standard treatments.Neonatal hepatoblastoma is rare so that it is necessary to conduct a multicenter and multidisciplinary study.