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
Received date: 2022-12-06
Online published: 2023-09-05
目的 探讨儿童G4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。
武跃芳 , 孙艳玲 , 武万水 , 杜淑旭 , 李苗 , 孙黎明 . G4型髓母细胞瘤患儿预后影响因素及生存状况分析[J]. 临床儿科杂志, 2023 , 41(9) : 686 -691 . DOI: 10.12372/jcp.2023.22e1634
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 Chr12p+ 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. Chr12p + may be related to the prognosis of children, but it needs to be confirmed by clinical studies with larger samples.
Key words： group 4 medulloblastoma; prognosis; influencing factor; child
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