21例脑膜旁横纹肌肉瘤中枢侵犯组患儿的临床特征及预后分析  

Clinical features and prognosis of 21 children with parameningeal rhabdomyosarcoma accompany with central nerve system invasion

作　　者：胡慧敏[1] 张伟令[1] 黄东生[1] 李静[1] 李凡[1] 支天[1] Hu Huimin;Zhang Weiling;Huang Dongsheng;Li Jing;Li Fan;Zhi Tian(Department of Pediatrics,Beijing Tongren Hospital,Capital Medical University,Beijing 100730,China)

机构地区：[1]首都医科大学附属北京同仁医院儿科,北京100730

出　　处：《中国医学前沿杂志（电子版）》2021年第12期60-65,共6页Chinese Journal of the Frontiers of Medical Science(Electronic Version)

基　　金：北京市医管局“登峰”人才培养计划(DFL20180201)。

摘　　要：目的总结儿童脑膜旁横纹肌肉瘤(parameningeal rhabdomyosarcoma,PM-RMS)中枢侵犯组患儿的临床特征和预后特点。方法回顾性分析首都医科大学附属北京同仁医院自2004年5月1日至2017年12月31日收治的21例明确诊断PM-RMS中枢侵犯组患儿的临床资料,应用统计学方法分析患儿的临床特点、综合治疗模式和预后情况。结果21例PM-RMS中枢侵犯组患儿,脑神经受累11例,颅底骨受累14例,脑脊液细胞学阳性18例;病理分型腺泡型6例,胚胎型15例;TNM分期3期16例,4期5例;美国横纹肌肉瘤研究组分组Ⅲ组3例,Ⅳ组18例;活检16例,行肿瘤部分切除手术5例;仅予以化疗10例,联合放化疗11例。截至2018年12月31日,随访17~136个月,中位随访时间44.5个月,1例患儿因病情进展放弃治疗后失访,有效随访20例。治疗过程中无复发/进展者7例,其中完全缓解6例,局部复发5例,疾病进展8例;死亡11例,存活9例。20例患儿的生存时间为(62.68±13.14)个月,5年总生存率为38.3%。联合放化疗患儿生存时间为(88.94±15.68)个月,5年总生存率显著高于单独化疗患儿(57.1%∶0,χ^(2)=9.954,P=0.002)。治疗后无复发/进展患儿5年总生存率显著高于复发/进展患儿(100%∶20.0%∶16.7%,χ^(2)=8.477,P=0.014)。结论儿童PM-RMS中枢侵犯组总体预后差,化疗联合放疗能显著改善患儿预后。Objective To summarize the clinical features and prognosis of children with parameningeal rhabdomyosarcoma(PM-RMS)accompany with central nerve system invasion.Method A retrospective analysis was performed on the clinical data of 21 children admitted to Beijing Tongren Hospital,Capital Medical University from May 1,2004 to December 31,2017 who were diagnosed with PM-RMS accompany with central nerve system invasion.The clinical features,comprehensive treatment mode and prognosis of the patients were analyzed by statistical methods.Result The 21 children were diagnosed by pathology,including 11 cases of cranial nerve involvement,14 cases of skull involvement,and 18 cases of cerebrospinal fluid cytology positive.Pathological classification was 6 cases alveolar type and 15 cases embryonal type.TNM staging included 16 cases with stage 3 and 5 cases with stage 4.The clinical groups according to Intergroup Rhabdomyosarcoma Study were 3 cases in groupⅢand 18 cases in groupⅣ.Biopsy was performed in 16 cases and partial tumor resection was performed in 5 cases.Only 10 cases received chemotherapy and 11 cases received combined chemoradiotherapy.As of December 31,2018,the follow-up period was 17 to 136 months,with a median follow-up time of 44.5 months.One child was lost to follow-up after giving up treatment due to disease progression,and 20 cases children were effectively followed up.There were 7 children with no recurrence/progression during treatment,including 6 children with complete remission,5 children with local recurrence and 8 children with disease progression.Eleven children died and nine survived.The survival time was(62.68±13.14)months,and the 5-year overall survival rate was 38.3%.The survival time of patients with combined chemoradiotherapy was(88.94±15.68)months,5-year overall survival rate of children with combined chemoradiotherapy was significantly higher than that of children with chemotherapy alone(57.1%∶0,χ^(2)=9.954,P=0.002).After treatment,the 5-year overall survival rate of children without r

关 键 词：横纹肌肉瘤 儿童 放疗 预后 脑膜旁横纹肌肉瘤 

分 类 号：R739.45[医药卫生—肿瘤]