儿童H3 K27M突变型弥漫性中线胶质瘤的MRI表现  

MRI Features of Pediatric Diffuse Midline Gliomas with H3 K27M Mutation

作　　者：马秋红[1] 周霞 李理 易婷 向永华[1] 金科[1] MA Qiuhong;ZHOU Xia;LI Li;YI Ting;XIANG Yonghua;JIN Ke(Department of Radiology,Hunan Children’s Hospital,Changsha 410007,China)

机构地区：[1]湖南省儿童医院放射科,湖南长沙410007

出　　处：《中国医学影像学杂志》2022年第5期435-439,464,共6页Chinese Journal of Medical Imaging

基　　金：湖南省卫生健康委2022年度科研计划项目(202209012935)

摘　　要：目的探讨儿童H3 K27M突变型弥漫性中线胶质瘤(DMG)的MRI表现,提高对本病的认识及诊断水平。资料与方法回顾性分析2018年9月—2021年5月湖南省儿童医院及外院经病理证实的15例H3 K27M突变型DMG患儿的临床及影像学资料,15例行MRI平扫,其中14例行增强扫描,10例行扩散加权成像检查,8例行磁敏感加权成像检查。分析肿瘤部位、形态、边界、瘤周水肿、大小、信号特点(有无囊变、出血)、扩散情况及强化特点。结果15例肿瘤主体均位于中线部位;9例形态不规则,6例呈类圆形;12例边界清晰,瘤周无水肿,3例边界模糊,瘤周轻中度水肿;肿瘤最大直径3.3~5.7 cm;15例病灶信号欠均匀,10例肿块内存在形态及大小不一的囊变,均呈偏心性改变,5例无囊变,1例出血。10例扩散加权成像示病变均呈不同程度扩散受限,最小表观扩散系数为(0.992±0.262)×10^(−3) mm^(2)/s。14例增强扫描后,5例呈环状强化,4例小斑片状轻度强化,2例结节状强化,2例轻度不均匀强化,1例无强化;8例有基底动脉包绕。结论儿童伴H3 K27M突变型DMG好发于脑桥等中线部位,瘤内易发生偏心性囊变,瘤周清晰多无水肿,多呈环状、结节状及小斑片状强化等,MRI特征具有一定的特异性。Purpose To investigate the MRI features of diffuse midline gliomas(DMG)with H3 K27M mutation in children,and to improve the understanding and diagnosis of this tumor.Materials and Methods The clinical and imaging data of 15 children with H3 K27M mutant DMG confirmed by pathology in Hunan Children's Hospital from September 2018 to May 2021 were retrospectively analyzed.All patients underwent MRI scan,14 with enhanced MRI,10 with diffusion weighted imaging,and 8 with susceptibility-weighted imaging.The location,shape,boundary,peritumoral edema,size,signal characteristics(cystic degeneration,hemorrhage or not),diffusion and enhancement of the tumor were observed and analyzed.Results The tumors in 15 patients were all located in the midline.The tumors were irregular in 9 cases and quasi-circular in 6 cases.Tumors showed clear boundary in 12 cases without obvious peritumoral edema,and blur boundary with mild to moderate edema in 3 cases.The maximum diameter of the tumor was 3.3-5.7 cm.The signal intensity of the tumor was heterogeneous in all 15 cases with eccentric cystic in 10 cases and no cystic degeneration in 5 cases.One lesion had hemorrhage.Diffusion restricted was found in 10 cases,and the minimum apparent diffusion coefficient value was(0.992±0.262)×10^(−3)mm^(2)/s.After enhancement,5 cases showed ring enhancement,4 cases showed small patchy mild enhancement,2 nodular enhancement,2 mild uneven enhancement,and 1 no enhancement.Basilar artery was surrounded by the rumors in 8 cases.Conclusion Diffuse midline gliomas with H3 K27M mutant are more likely to develop in midline sites such as pons in pediatric patients.Characteristic MR performances include eccentric cyst in the lesion,clear boundary with little peritumoral edema,as well as ring,nodular or patchy enhancement.

关 键 词：H3 K27M突变 弥漫性中线胶质瘤 磁共振成像 扩散加权成像 磁敏感加权成像 儿童 

分 类 号：R739.41[医药卫生—肿瘤] R445.2[医药卫生—临床医学]