目的 分析儿童肝脏未分化胚胎性肉瘤（UESL）的临床特征、治疗方案及预后。方法 回顾分析2012年11 月至2019年6月收治的8例UESL患儿的临床资料，采用Kaplan-Meier生存分析法计算生存率。结果 8例患儿中，男性5 例、女性3例，中位发病年龄7岁（8个月~ 10岁）；8例患儿的肿瘤均位于肝右叶，中位最大径为13 . 5 cm（10 ~ 20 cm），超声 检查均显示为混合回声为主的肝内囊实性肿块。所有患儿手术完整切除肿块，3例术前肿瘤破裂。术后均接受顺铂/长春 新碱/阿霉素/环磷酰胺（AVCP）方案与异环磷酰胺/依托泊苷/长春新碱（IEV）交替化疗6个周期。患儿中位随访时间47 （24~90）月，7例完全缓解，1例死于复发；5年无事件生存率（EFS）为（79.25±9 . 31）%。结论 UESL患儿术前可出现肿 瘤破裂，完整切除手术联合化疗能获得长期无病生存，少数复发患儿预后不佳。

Objective To analyze the clinical features, treatment and prognosis of undifferentiated embryonal sarcoma of the liver (UESL) in children. Methods The clinical data of 8 children with UESL admitted from November 2012 to June 2019 were retrospectively analyzed. Kaplan-Meier survival analysis was used to calculate the survival rate. Result There were 8 children ( 5 boys and 3 girls). The median age of onset was 7 years ( 8 months to 10 years). The tumors of the 8 children were all located in the right lobe of the liver, and the median maximum diameter was 13 . 5 cm ( 10 - 20 cm). Ultrasonography showed intrahepatic solid cystic mass with mixed echogenicity. All patients underwent complete resection of the tumor, and 3 patients had tumor rupture before surgery. Six cycles of alternating chemotherapy were performed between AVCP regimen (cisplatin, vincristine, adriamycin and cyclophosphamide) and IEV regimen (ifosfamide, etoposide and vincristine). The median follow-up time was 47 ( 24 - 90 ) months, 7 children were in complete remission, and 1 died of recurrence. The 5 -year event free survival (EFS) was ( 79 . 25±9 . 31 ) %. Conclusions Preoperative tumor rupture may occur in children with UESL. Complete resection combined with chemotherapy can achieve long-term disease-free survival.