摘要
目的分析间变性室管膜瘤(EP)患儿的预后影响因素。方法2015年7月—2018年7月期间确诊的间变性EP患儿,共63例,病理明确为间变性室管膜瘤,分子分型为幕上室管膜瘤RELA基因融合突变型(ST-EPN-RELA)或后颅窝室管膜瘤A型(PF-EPN-A)。采用Kaplan-Meier法统计患儿的3年无进展生存(PFS)率和总体生存(OS)率,两组间比较采用Log-Rank分析。结果截止至2020年9月1日,中位随访时间3.0年(范围0.6~7.6年),初治患儿35例,复发28例。初治患儿的3年PFS率和OS率分别为(73.3±7.7)%和(78.7±7.7)%;复发患儿的3年PFS率和OS率分别为(7.1±4.9)%和(70.9±4.9)%,差异具有显著性(P<0.05)。年龄和发病部位不影响患儿的PFS和OS;手术全切和术后及早接受放射治疗可显著提高患儿的生存率(P<0.05);化疗可显著改善患儿的PFS和OS(P<0.05)。单纯化疗患儿(4例)生存率最低,2年OS为(25.0±21.7)%;初诊时年龄≤3岁的患儿在化疗后接受延迟放疗(22例),其3年PFS为(66.2±12.5)%,3年OS达(90.9±8.7)%;初诊时年龄>3岁、放疗后按序接受化疗的患儿(37例),其3年PFS较低,为(37.3±7.8)%,但OS有所提高,可达(77.3±6.8)%。所有患儿未出现放射相关性损伤,但是出现不同程度的骨髓抑制和胃肠道不良反应,对症处理后很快好转。结论儿童间变性EP预后差,手术及放射治疗是其主要治疗手段。低年龄室管膜瘤患儿接受化学治疗,可适当延迟放疗,提高生存率;复发患儿即使化学治疗疗效不显著,也可以适当延长生存期。
Objective To analyze the prognostic factors of anaplastic ependymoma(EP)in children.Methods Clinical data of 63 children with anaplastic EP diagnosed between July 2015 and July 2018 were analyzed.All cases were pathologically confirmed as anaplastic EP,and the molecular types were supratentorial ependymoma with RELA gene fusion(ST-EPN-RELA)or posterior fossa ependymoma type A(PF-EPN-A).The 3-year progression-free survival(PFS)rates and overall survival(OS)rates were calculated by Kaplan-Meier method.Log Rank analysis was used in the comparison among different groups,P<0.05 was defined as statistically significant.Results As of September 2020,the median follow-up time was 3.0(range 0.6 to 7.6)years,35 cases were newly treated and 28 cases were recurrent.The 3-year PFS rate and OS rate of newly treated patients were(73.3±7.7)%and(78.7±8.1)%,the 3-year PFS rate and OS rate of recurrent patients were(7.1±4.9)%and(70.9±8.8)%,the differences between newly treated and recurrent patients were significant(P<0.05).PFS and OS rates were not related with age and tumor location.Total resection and early postoperative radiotherapy can significantly improve the survival rate of children(P<0.05).Chemotherapy can significantly improve PFS and OS rates(P<0.05).The survival rate was the lowest in 4 patients treated with chemotherapy alone,and the 2-year OS of them was(25.0±21.7)%.The 3-year PFS and OS rates were(66.2±12.5)%and(90.9±8.7)%in 22 children(≤3 years old at first diagnosis)who received delayed radiotherapy after chemotherapy.The 3-year PFS rate of 37 children(>3 years old at first diagnosis)who received chemotherapy in sequence after radiotherapy was only(37.3±7.8)%,but the OS rate improved to(77.3±6.8)%.All the children did not have radiation-related injuries,but showed different degrees of bone marrow suppression and gastrointestinal adverse reactions,which were soon improved after symptomatic treatment.Conclusions The prognosis of children with anaplastic EP is poor,surgery and radiotherapy are main treatmen
作者
李舒婷
高文超
杜淑旭
张金
刘妍
王圆
龚小军
孙艳玲
武万水
孙黎明
LI Shuting;GAO Wenchao;DU Shuxu;ZHANG Jin;LIU Yan;WANG Yuan;GONG Xiaojun;SUN Yanling;WU Wanshui;SUN Liming(Beijing Shijitan Hospital of Capital Medical University, Beijing 100038, China)
出处
《中国小儿血液与肿瘤杂志》
CAS
2021年第4期218-222,共5页
Journal of China Pediatric Blood and Cancer
关键词
室管膜瘤
儿童
生存
化疗
Ependymoma
Children
Survival rate
Chemotherapy
