儿童弥漫性中线胶质瘤伴H3 K27M突变MRI表现  

MRI characteristics of diffuse midline gliomas with H3 K27M mutation in children

作　　者：丁茗[1] 郑慧[1] 冯赟[1] 李芳珍[1] 尹秋凤[1] 张玉珍[1] 李玉华[1] 汪登斌[1] 蔡静[1] DING Ming;ZHENG Hui;FENG Yun;LI Fangzhen;YIN Qiufeng;ZHANG Yuzhen;LI Yuhua;WANG Dengbin;CAI Jing(Department of Radiology,Xinhua Hospital Affiliated to Shanghai Jiaotong University School of Medicine,Shanghai 200092,China)

机构地区：[1]上海交通大学医学院附属新华医院放射科,上海200092

出　　处：《实用放射学杂志》2020年第3期444-447,共4页Journal of Practical Radiology

基　　金：国家重点研发计划课题(2017YFC0109003)。

摘　　要：目的探讨儿童弥漫性中线胶质瘤伴H3 K27M突变的MRI表现及其鉴别诊断。方法回顾性分析经手术病理证实的37例儿童弥漫性中线胶质瘤伴H3 K27M突变的临床及MRI表现。结果34例病灶位于脑干,2例位于丘脑,1例位于脊髓。脑干肿瘤边界不清、浸润生长,呈T1WI稍低、T2 FLAIR高信号。31例扩散未受限。13例内见小囊变,5例内见大片囊变区。增强后强化方式不一,11例未见强化区,10例环形强化,7例小结节样强化。2例丘脑肿瘤均呈浸润性生长,平扫信号均匀,扩散受限,增强后无强化。1例脊髓肿瘤边界不清,增强后环形强化。结论儿童弥漫性中线胶质瘤伴H3 K27M突变多数位于脑干,呈浸润性生长,强化范围不一,扩散多不受限。丘脑肿瘤扩散受限,无强化,脊髓肿瘤环形强化。Objective To investigate the MRI features and antidiastole of diffuse midline gliomas with H3 K27M mutation in children.Methods The clinical and MRI findings of 37 children were analyzed retrospectively,who were confirmed as diffuse midline gliomas with H3 K27M mutation by surgery and pathology.Results 34 cases located in brainslem,2 cases in thalamus and 1 case in spinal cord.Tumors in brainstem had infiltrating growth and obscure margin,showing slight hypointensity on T1WI and hyperintensily on T2FLAR.31 of them had no restricted diffusion.In addition,small cystic region were found in 13 cases and big in 5 cases.After administrati ng contrast age nt,they had varies enhancement patterns:11 cases no enha need,10 cases circularly enhanced and 7 cases nodular enhanced.2 thalamic tumors manifested as infiltrative masses with homogeneous intensity,limited diffusion,and no enhancement.1 spinal cord tumor showed a poorly defined lesion with rim enhancement.Conclusion Diffuse midline gliomas with H3 K27M mutation in children mostly located in brainstem,and appeared as infiltrative masses with variable enhancement ranges and no restricted diffusion.The thalamic tumors showed restricted diffusion without enhancement and the tumors in spinal cord manifestated as rim enhancement.

关 键 词：儿童 弥漫性中线胶质瘤 H3 K27M突变 

分 类 号：R739.41[医药卫生—肿瘤] RQ344+.12[医药卫生—临床医学]