单中心87例髓母细胞瘤的预后因素分析  

Survival prognosis analysis of 87 children with medulloblastoma in a single center

作　　者：杨伟 葛明[1] 孙骇浪[1] 蔡英杰 冀园琦[1] 彭小娇 赵凤毛[1] 张天蕾 杨萍 陈佳树 Yang Wei;Ge Ming;Sun Hailang;Cai Yingjie;Ji Yuanqi;Peng Xiaojiao;Zhao Fengmao;Zhang Tianlei;Yang Ping;Chen Jiashu(Department of Neurosurgery,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China)

机构地区：[1]国家儿童医学中心,首都医科大学附属北京儿童医院神经外科,北京100045

出　　处：《中华神经外科杂志》2023年第1期2-7,共6页Chinese Journal of Neurosurgery

基　　金：北京市医院管理中心临床医学发展专项(XMLX202144)。

摘　　要：目的探讨影响髓母细胞瘤(MB)患儿生存预后的因素。方法回顾性分析2012年12月至2019年12月首都医科大学附属北京儿童医院神经外科收治的87例MB患儿的临床资料。87例患儿均行手术切除肿瘤,术后69例行化疗,73例行放疗。采用Kaplan-Meier法分析患儿的总生存期(OS)。采用单因素log-rank检验和多因素Cox回归分析探讨患儿OS的影响因素。结果87例患者中,75例达肿瘤全切除,10例为近全切除,2例行部分切除。术后组织病理学结果表明,经典型64例,促纤维增生/结节型13例,大细胞/间变型6例,广泛结节型4例;分子分型结果显示,32例患儿中,Group 4型17例,SHH型10例,Group 3型和WNT型分别为3例和2例。术后诊断为小脑性缄默综合征(CMS)者33例(37.9%)。87例患儿的中位随访时间(范围)为22.5(1~86)个月,7年生存率为61.7%。单因素log-rank分析结果提示,危险分层(χ^(2)=15.80,P<0.001)、术前存在肿瘤播散(χ^(2)=13.72,P<0.001)、术后放疗(χ^(2)=25.92,P<0.001)、术后化疗(χ^(2)=3.89,P=0.049)与MB患儿的OS可能有关;多因素Cox回归分析结果显示,仅术后放疗为MB患儿OS的独立影响因素(HR=-2.37,95%CI:0.03~0.31,P<0.001),而危险分层、术后化疗、年龄、肿瘤切除程度、术前有无肿瘤播散、术后是否诊断为CMS均非OS的影响因素(均P>0.05)。结论术后是否发生CMS并不影响MB患儿的生存预后,而术后放疗有利于改善其预后。Objective To explore the factors affecting the survival prognosis of children with medulloblastoma(MB).Methods The clinical data of 87 children with MB admitted to the Neurosurgery Department of Beijing Children′s Hospital,Capital Medical University from December 2012 to December 2019 were retrospectively analyzed.All 87 cases underwent tumor resection,69 cases received chemotherapy and 73 cases received radiotherapy.The overall survival(OS)of the children was analyzed by Kaplan-Meier method.Univariate log-rank test and multivariate Cox regression analysis were used to explore the influencing factors of OS in children.Results Among the 87 cases of medulloblastoma,gross total resection was achieved in 75 cases,near total resection in 10,and partial resection in 2.Postoperative pathological results revealed 64 cases of classic type,13 cases of desmoplastic/nodular type,6 cases of large cell/anaplastic type and 4 cases of extensive nodularity type.According to the molecular typing method and out of 32 cases,there were 17 cases of Group 4,10 cases of SHH type,3 cases of Group 3 type and 2 cases of WNT type.Post operation,33 cases(37.9%)were diagnosed with cerebellar mutism syndrome(CMS).The follow-up time was 1-86 months with a median of 22.5 months.The overall 7-year survival rate was 61.7%.Kaplan-Meier analysis results showed that risk hierarchy(χ^(2)=15.80,P<0.001),preoperative tumor dissemination(χ^(2)=13.72,P<0.001),postoperative radiotherapy(χ^(2)=25.92,P<0.001)and postoperative chemotherapy(χ^(2)=3.89,P=0.049)were associated with OS.Multivariate Cox regression analysis showed that postoperative radiotherapy was the only independent risk factor affecting OS(HR=-2.37,95%CI:0.03-0.31,P<0.001),while risk hierarchy,preoperative tumor dissemination,age,postoperative chemotherapy,or CMS were not risk factors of OS(all P>0.05).Conclusion Postoperative CMS does not affect the prognosis of MB,and postoperative radiotherapy can help improve the prognosis of MB.

关 键 词：髓母细胞瘤 儿童 小脑性缄默综合征 生存分析 危险因素 

分 类 号：R739.4[医药卫生—肿瘤]