儿童肝脏未分化胚胎性肉瘤8例临床分析及文献复习  

Clinical analysis of eight children with undifferentiated embryonal sarcoma of the liver in children and literature review

作　　者：唐京京[1] 马怡晖[2] 徐学聚[1] 白松婷[1] 王璐[1] 王颖超[1] 王春美[1] 尼晓丽 刘玉峰[1] TANG Jingjing;MA Yihui;XU Xueju;BAI Songting;WANG Lu;WANG Yingchao;WANG Chunmei;NI Xiaoli;LIU Yufeng(Department of Hematology and Oncology,Children’s Hospital,The First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,Henan,China;Department of Pathology,Children’s Hospital,The First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,Henan,China)

机构地区：[1]郑州大学第一附属医院儿童医院血液肿瘤科,河南郑州450052 [2]郑州大学第一附属医院病理科,河南郑州450052

出　　处：《临床儿科杂志》2021年第10期758-760,共3页Journal of Clinical Pediatrics

摘　　要：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.

关 键 词：肝 胚胎性肉瘤 预后 儿童 

分 类 号：R735.7[医药卫生—肿瘤]