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G4型髓母细胞瘤患儿预后影响因素及生存状况分析

Analysis of prognostic factors and survival status of group 4 medulloblastoma in children
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摘要 目的探讨儿童G 4型髓母细胞瘤(MB)患者的生存状况及其预后影响因素。方法回顾性分析2016年5月至2020年8月儿科收治的G4型MB患儿(随访至2022年8月)的临床资料,采用Kaplan-Meier法计算总体生存(OS)率和无进展生存(PFS)率,采用Log-rank检验比较组间生存率差异,采用Cox回归模型分析影响预后的因素。结果纳入145例G4型MB患儿,男106例、女39例,中位确诊年龄7.5(5.7~9.6)岁。M0期91例,M+期54例(M1期1例、M2期12例、M3期41例);病理分型为经典型127例、促纤维增生/结节型(DN)8例、大细胞/间变型(LC/A)8例、广泛结节型(EN)1例、未分型1例。中位随访时间为47.9(36.5~59.3)月,复发37例。患儿5年OS率和PFS率分别为(80.8±3.4)%和(55.4±4.7)%。Cox回归分析结果提示M+期、MYCN扩增、Chr12 p+变异是影响预后的独立危险因素(P<0.05)。结论M+期、MYCN扩增的G4型MB患儿预后相对较差。Chr12 p+可能与患儿预后相关,但仍有待更大样本的临床研究进行证实。 Objective To investigate the survival status and prognostic factors of group 4 medulloblastoma(MB)in children.Methods The clinical data of children with group 4 MB admitted to the Department of Pediatrics from May 2016 to August 2020(follow-up until August 2022)were retrospectively analyzed.The Kaplan-Meier method was used to calculate the overall survival(OS)rate and progression-free survival(PFS)rate.Log-rank test was used to compare the difference in survival rate between groups,and Cox regression model was used to analyze the factors affecting prognosis.Results A total of 145 children(106 boys and 39 girls)with group 4 MB were included,and the median age of diagnosis was 7.5(5.7-9.6)years old.There were 91 children in M0 stage and 54 children in M+stage(1 in M1 stage,12 in M2 stage and 41 in M3 stage).The pathologic types were classic in 127 cases,desmoplastic/nodular(DN)in 8 cases,anaplastic/large cell(LC/A)in 8 cases,extensive nodularity(EN)in 1 case,and none of somatotype(NOS)in 1 case.The median follow-up time was 47.9(36.5-59.3)months,and 37 children had tumor recurrence.The 5-year OS and PFS rates were(80.8±3.4)%and(55.4±4.7)%,respectively.Cox regression analysis indicated that M+stage,MYCN amplification and Chr 12 p+variation were independent risk factors for prognosis(P<0.05).Conclusions Group 4 MB children with M+stage or MYCN amplification have a relatively poor prognosis.Chr 12 p+may be related to the prognosis of children,but it needs to be confirmed by clinical studies with larger samples.
作者 武跃芳 孙艳玲 武万水 杜淑旭 李苗 孙黎明 WU Yuefang;SUN Yanling;WU Wanshui;DU Shuxu;LI Miao;SUN Liming(Department of Pediatrics,Beijing Shijitan Hospital,Capital Medical University,Beijing 100038,China)
出处 《临床儿科杂志》 CAS CSCD 北大核心 2023年第9期686-691,共6页 Journal of Clinical Pediatrics
关键词 G4型髓母细胞瘤 预后 影响因素 儿童 group 4 medulloblastoma prognosis influencing factor child
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