儿童初治肝母细胞瘤83例临床特征及预后分析

目的探讨肝母细胞瘤(HB)患儿的临床特征、生存情况及预后危险因素。方法回顾性分析2012年1月至2019年10月郑州大学第一附属医院儿童医院血液肿瘤科收治的83例初治HB患儿的临床资料, 记录患儿的性别、年龄、首发临床表现、治疗前病变范围(PRETEXT)分期、病理类型、病初甲胎蛋白(AFP)、治疗方法及治疗结果。2018年之前确诊的患儿采用"武汉方案"治疗, 2018年之后确诊的患儿采用"儿童肝母细胞瘤多学科诊疗专家共识(CCCG-HB-2016)"方案治疗。Kaplan-Meier生存分析法计算生存率, 单因素分析采用Log-Rank检验, 多因素预后分析采用Cox回归模型。结果 83例患儿中男51例、女32例;发病年龄25.2(9.0, 34.0)月龄, <3岁64例(77%)。最常见的首发临床表现为腹部包块(45例, 54%)。PRETEXT Ⅰ期8例, Ⅱ期43例, Ⅲ期20例, Ⅳ期12例。随访时间40(17, 63)个月, 全组HB患儿1年总体生存率(OS)和无事件生存率(EFS)分别为(84±4)%和(79±5)%, 5年OS和EFS分别为(78±5)%和(76±...

Clinical characteristics and prognosis of 83 children with newly treated hepatoblastoma

Objective To investigate the clinical features,survival and prognostic risk factors of children with hepatoblastoma(HB).Methods Clinical data of 83 children with newly treated HB at the Department of Hematology and Oncology,Children′s Hospital,the First Affiliated Hospital of Zhengzhou University from January 2012 to October 2019 were analyzed retrospectively.The sex,age,first clinical manifestations,pretreatment extent of disease(PRETEXT)stages,pathological types,initial alpha-fetoprotein(AFP),treatment methods and treatment outcome of all patients were summarized.The children diagnosed before 2018 were treated with"Wuhan Protocol",and those who diagnosed after 2018 were treated with the"Expert Consensus for Multidisciplinary Management of Hepatoblastoma"(CCCG-HB-2016)protocol.Kaplan-Meier survival analysis was used to calculate the survival rate,Log-Rank test was used in univariate analysis,and the Cox regression model was used in multivariate prognosis analysis.Results Among 83 cases,there were 51 males and 32 females.The age of onset was 25.2(9.0,34.0)months old,and 64 cases(77%)were under 3 years old.The most common first clinical manifestation was abdominal mass in 45 cases(54%).There were 8 cases of PRETEXT stageⅠ,43 cases of stageⅡ,20 cases of stageⅢand 12 cases of stageⅣ.During the follow-up period of 40(17,63)months,the 1-year overall survival(OS)rate and event-free survival(EFS)rate were(84±4)%and(79±5)%,respectively,and 5-year OS rate and EFS rate were(78±5)%and(76±5)%,respectively.Fifty-five cases were treated with"Wuhan Protocol",and the 5-year OS and EFS rate were(73±6)%and(71±6)%,respectively.Twenty-eight cases were treated with CCCG-HB-2016 protocol,and the 5-year OS and EFS rate were(88±7)%and(82±9)%,respectively.Multivariate COX regression analysis showed that AFP did not turn negative after 3 courses of postoperative chemotherapy(HR=9.228,95%CI 1.017-83.692)and PRETEXT stageⅣ(HR=6.587,95%CI 1.687-25.723)were independent risk factors affecting the prognosis of children with HB.Conclusions The"Wuhan Protocol"and CCCG-HB-2016 protocol were effective in the treatment of children with HB.AFP did not turn negative after 3 courses of postoperative chemotherapy and PRETEXT stageⅣwere independent risk factors affecting the prognosis of children with HB.