髓母细胞瘤术后发生小脑性缄默综合征患儿的生存预后分析

Survival and prognosis analysis of children with cerebellar mutism syndrome after medulloblastoma surgery

目的探讨术后发生小脑性缄默综合征(CMS)的髓母细胞瘤患儿的生存预后及其影响因素。方法回顾性分析2013年4月至2018年6月于首都医科大学附属北京天坛医院神经外科学中心接受手术切除治疗的104例髓母细胞瘤患儿的临床资料。将所有患儿按照术后是否发生CMS分为两组,并将术后发生CMS的患儿分为静默型和经典型CMS两组。依据2006年儿童肿瘤协作组提出的小脑缄默调查表,对发生CMS患儿缄默症状的严重程度进行分级评估。采用Kaplan-Meier法计算患儿的总生存率(OS)和无进展生存率(PFS),以log-rank法比较不同分组间的生存差异。采用单因素和多因素Cox回归分析探讨发生CMS的髓母细胞瘤患儿生存预后的影响因素。结果果截至2020年6月,104例患儿的中位随访时间(范围)为52个月(24~107个月);78例(75.0%)存活,26例(25.0%)死亡;36例(34.6%)肿瘤复发。术后发生CMS共40例(38.5%),其中静默型9例,经典型31例。经典型CMS患儿较静默型CMS患儿在缄默失语及易怒表现评分中的得分更高(均P<0.05)。是否发生CMS对髓母细胞瘤患儿5年PFS及OS影响的差异均无统计学意义(均P>0.05)。静默型CMS患儿的5年PFS高于经典型CMS患儿(P=0.047),而两者5年OS的差异无统计学意义(P=0.165)。多因素Cox回归分析结果显示,术前肿瘤播散(HR=9.91,95%CI:2.37~41.43,P=0.002)和肿瘤是否侵犯小脑中脚(HR=0.13,95%CI:0.03~0.54,P=0.005)均为发生CMS患儿PFS的独立影响因素。结论CMS是儿童髓母细胞瘤术后常见的并发症,术后是否发生CMS并不影响患儿的生存预后。静默型CMS患儿的症状较经典型CMS患儿更轻,生存预后相对更佳。此外,术前肿瘤播散和肿瘤侵犯小脑中脚为影响术后发生CMS患儿生存预后的不利因素.

Objective To investigate the survival and prognosis of children with postoperative cerebellar mutism syndrome(CMS)after resection of medulloblastoma and the influencing factors.Methods A retrospective analysis was conducted on the clinical data of 104 children with medulloblastoma who underwent surgical resection in the Neurosurgery Center of Beijing Tiantan Hospital,Capital Medical University from April 2013 to June 2018.All children were divided into two groups according to whether postoperative CMS occurred.Based on the different symptomological manifestations,children with postoperative CMS were divided into silent CMS group and classic CMS group.According to the CMS survey proposed by the Children's Oncology Group in 2006,the severity of mutism symptoms in children with CMS was graded and evaluated.The Kaplan-Meier method was used to calculate the overall survival(OS)and progression-free survival(PFS)of the children,and the log-rank analysis was used to compare the survival differences between different groups.Univariate and multivariate Cox regression analysis were used to investigate the factors influencing the survival and prognosis of children with medulloblastoma who developed CMSR.esultsAs of June 2020,the median follow-up time of 104 children was 52 months(24-107 months).A total of 78 cases(75.0%)survived,26 cases(25.0%)died and 36 cases(34.6%)had tumors relapse.A total of 40 cases(38.5%)developed CMS,including 9 cases of silent type and 31 cases of classic type.Compared with children with silent CMS,children with classic CMS had higher scores in the scores of mutism and irritability(both P<0.05).There was no significant difference in the 5-year PFS and OS of children with medulloblastoma whether CMS occurred or not(both P>0.05).The 5-year PFS of children with silent CMS was higher than that of children with classic CMS(P=0.047),but there was no significant difference in 5-year OS between the two groups(P=0.165).Multivariate Cox regression analysis showed that tumor dissemination occurring before operation(HR=9.91,95%CI:2.37-41.43,P=0.002)and whether the tumor invaded the middle cerebellar peduncle(HR=0.13,95%CI:0.03-0.54,P=0.005)were independent influencing factors of PFS in children with CMS.Conclusions CMS is a common complication after surgery for medulloblastoma in children,and whether CMS occurs after surgery does not affect the survival prognosis of children.Among them,children with silent CMS have milder symptoms than children with classic CMS,and their survival prognosis is relatively better.In addition,preoperative tumor dissemination and tumor invasion of the middle cerebellar peduncle are unfavorable factors affecting the survival and prognosis of children with postoperative CMS.