儿童脑干胶质瘤的临床特点及预后分析  

Clinical features and surgical prognosis in children with brain stem glioma

作　　者：葛明[1] 杨伟 李少武[2] 蔡英杰 孙骇浪[1] 冀园琦[1] 张迪[1] 彭小娇 王佳 Ge Ming;Yang Wei;Li Shaowu;Cai Yingjie;Sun Hailang;Ji Yuanqi;Zhang Di;Peng Xiaojiao;Wang Jia(Department of Neurosurgery,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China;Beijing Institute of Neurosurgery,Capital Medical University,Beijing 100070,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,100045 [2]首都医科大学,北京市神经外科研究所,100070

出　　处：《中华神经外科杂志》2021年第7期679-683,共5页Chinese Journal of Neurosurgery

摘　　要：目的:总结儿童脑干胶质瘤(BSG)的临床特点及预后。方法:回顾性分析2013年5月至2020年12月首都医科大学附属北京儿童医院神经外科采用手术治疗的19例BSG患儿的临床资料,其中对肿瘤未全切除者术后行放疗和(或)化疗。根据世界卫生组织(WHO)肿瘤分级将患儿分为低级别BSG组13例(WHOⅠ~Ⅱ级,简称低级别组)和高级别BSG组6例(均为Ⅳ级,简称高级别组)。分析儿童BSG的临床特点及生存情况,比较两组患儿的差异。体能状态评估采用美国东部肿瘤协作组(ECOG)的评分标准。结果:19例患儿男、女比例为12/7;中位诊断年龄为4.5岁,中位病程为1.0个月。术前13例患儿出现脑神经功能损害的临床表现。病理学结果显示,高级别组6例均为伴H3K27M突变的弥漫性中线胶质瘤;低级别组13例中,神经节细胞胶质瘤6例,星形细胞瘤7例。高级别组与低级别组比较,患儿的中位诊断年龄(分别为4.5、7.0岁)、肿瘤直径[分别为(3.2±0.8)、(2.2±0.9)mm]、手术全切除比例(分别为0/6、10/13),以及手术前、后ECOG评分中位数(术前分别为2.0、1.0分,术后分别为4.5、0分)差异均有统计学意义(均P<0.05)。高级别组6例肿瘤均为内生型,低级别组外生型12例,内生型1例,差异有统计学意义(P<0.05)。13例低级别组患者的中位随访时间为8个月,患儿均存活。高级别组5例死亡,存活时间为0.5~8.0个月;1例随访至8个月仍存活。结论:脑神经功能损害是儿童BSG最常见的临床表现,与低级BSG患儿比较,高级别BSG患儿的诊断年龄大、肿瘤体积大、以内生型为主、病程短、预后差。Objective To summarize the clinical characteristics and prognosis of brainstem glioma(BSG)in children.Methods The clinical data of 19 children with BSG who underwent surgical treatment in Department of Neurosurgery,Beijing Children′s Hospital of Capital Medical University from May 2013 to December 2020 were retrospectively analyzed.Patients undergoing partial tumor excision received chemotherapy and/or radiotherapy after surgery.According to the WHO tumor classification,those children were divided into low-grade BSG group(WHO gradeⅠ-Ⅱ;13 cases)and high-grade BSG group(WHO gradeⅣ;6 cases).The clinical characteristics and survival of BSG children were analyzed,and the differences between the two groups were analyzed.The patient′s physical status was assessed using the scoring standard of the Eastern Cooperative Oncology Group(ECOG).Results The ratio of male to female in the 19 cases was 12/7.The median age at diagnosis was 4.5 years,and the median duration of disease was 1.0 month.Cranial nerve function damage occurred in 13 children before operation.The pathological results showed that the 6 cases in the high-grade group were all diffuse midline gliomas with H3K27M positive;among the 13 cases in the low-grade group,there were 6 cases of ganglioglioma and 7 cases of astrocytoma.Comparing the high-grade group with the low-grade group,the median age at diagnosis(4.5 years vs.7.0 years),tumor diameter(3.2±0.8 mm vs.2.2±0.9 mm),ratio of total surgical resection(0/6 vs.10/13),as well as the median pre-and post-operative ECOG scores(2.0,1.0 points vs.4.5,0 points)were statistically significant(all P<0.05).The 6 cases in the high-grade group were all endogenous;12 in the low-grade group were exogenous and 1 was endogenous.The difference was statistically significant(P<0.05).The median follow-up time of 13 cases in the low-grade group was 8 months,and all the children survived.In the high-grade group,5 patients died with a survival time of 0.5 to 8.0 months;1 patient was still alive after 8 months of follow-up.

关 键 词：脑干肿瘤 神经胶质瘤 儿童 疾病特征 预后 

分 类 号：R739.41[医药卫生—肿瘤]