摘要
目的分析小儿弥漫性中线胶质瘤H3K27M改变型(DMG-A)影像学特征。方法回顾性分析2019年2月~2023年10月本院收治的22例DMG-A患儿的病例资料。由2位工作10年以上经验丰富的儿科影像诊断医生进行独立阅片,观察病变部位、形态、大小、实性部分密度/信号特征、信号强度、弥散受限情况及MRS表现、瘤内钙化、出血及坏死/囊变、强化特点、瘤内是否有血管穿行、瘤周水肿、肿瘤毗邻及继发改变、有无远处转移以及转移部位等。结果CT检查10例,脑干病灶多为低/稍低密度影(9例),其次为等或稍高密度影(各1例)。表现为脑干增粗,多以脑桥为中心,部分累及延髓、中脑、小脑脚及小脑半球。丘脑病灶为团状不均匀稍高密度影(1例),并向环池及中脑右侧延伸。MRI检查21例,脑干病变表现为脑干明显增粗膨大,以桥脑为著,主要为T1WI低、T2WI/FLAIR稍高信号影(15例)。DWI可弥散受限(9例),也可无弥散受限(5例),另外1例无DWI检查。病灶多包绕基底动脉(14例)。丘脑病灶表现为丘脑体积增大(6例),T1WI主要为低信号影(5例),其次为等信号影(1例),T2WI/FLAIR均为稍高信号影。DWI可弥散受限(4例),也可不受限(2例)。部分向下累及大脑脚、中脑等。增强后多呈明显结节状、花环状及斑片状强化(13例),也可呈轻度不均匀强化和无明显强化(各3例),另外2例无MRI增强检查。MRS检查9例,主要表现为Cho、Cr峰升高,NAA峰减低,Cho+Cr/NAA升高。结论虽然儿童DMG-A的影像学表现多样,但仍具有一定的特征性,综合分析其多发年龄、发病部位、弥散有无受限和强化方式及范围等情况,有助于提高对该病影像学诊断与鉴别诊断水平。
Objective To analyze the imaging features of pediatric diffuse midline glioma H3K27M variant(DMG-A).Methods The data of 22 children with DMG-A admitted to our hospital from February 2019 to October 2023 were retrospectively analyzed.Preoperative CT scan was performed in 12 cases(11 cases simultaneously with MRI).The images were independently read by two experienced pediatric imaging diagnostic doctors with over 10 years of working experience.Observed the lesion location,shape,size,density/signal characteristics of the solid part,signal intensity,restricted diffusion and MRS Manifestations,intratumoral calcification,hemorrhage and necrosis/cystic changes,enhancement features,whether there are blood vessels passing through the tumor,peritumoral edema,adjacent and secondary changes of the tumor,whether there is distant metastasis,and the metastatic site,etc.Results CT examinations were conducted in 10 cases.The brainstem lesions were mostly low/slightly low density shadows(9 cases),followed by equal or slightly high density shadows(1 case each).It is manifested as thickening of the brainstem,mostly centered on the pons,and partially involving the medulla oblongata,midbrain,cerebellar peduncle and cerebellar hemispheres.The thalamic lesion was a mass-like,uneven,slightly high-density shadow(1 case),extending towards the cisterns and the right side of the midbrain.MRI examination was conducted in 21 cases.The brainstem lesions were characterized by significant thickening and enlargement of the brainstem,with the pons being the most prominent.The main signal shadows were low on T1WI and slightly high on T2WI/FLAIR(15 cases).DWI could have diffuse limitation(9 cases),or no diffuse limitation(5 cases),and another case had no DWI examination.The lesions mostly surrounded the basilar artery(14 cases).Thalamic lesions were manifested as an increase in thalamic volume(6 cases),mainly low signal shadows on T1WI(5 cases),followed by isosignal shadows(1 case),and slightly high signal shadows on T2WI/FLAIR.DWI may have diffuse limitation(4 cases)or no limitation(2 cases).It partially affects the cerebellum,midbrain and other parts downward.After enhancement,most cases presented with obvious nodular,flower-shaped and patchy enhancement(13 cases),and could also show mild heterogeneous enhancement and no obvious enhancement(3 cases each).Additionally,2 cases had no MRI enhancement examination.Nine cases underwent MRS Examination.The main manifestations were elevated Cho and Cr peaks,decreased NAA peak,and elevated Cho+Cr/NAA.Conclusion Although the imaging manifestations of DMG-A in children are varied,they still have certain characteristics.A comprehensive analysis of the age of occurrence,location of onset,whether the diffusion is limited,and the way and extent of intensification is helpful to improve the imaging diagnosis and differential diagnosis of the disease.
作者
杨春兰
曹娟
刘龙平
徐守军
YANG Chunlan;CAO Juan;LIU Longping;XU Shoujun(Department of Hematology and Oncology,Shenzhen Children's Hospital,Shenzhen 518026,China;Department of Pathology,Shenzhen Children's Hospital,Shenzhen 518026,China;Department of Radiology,Shenzhen Children's Hospital,Shenzhen 518026,China)
出处
《分子影像学杂志》
2025年第9期1085-1092,共8页
Journal of Molecular Imaging
基金
广东省高水平临床重点专科项目(SZGSP012)
深圳市医学重点学科建设经费项目(SZXK034)。
关键词
儿童
弥漫性中线胶质瘤
体层摄影术
X线计算机
磁共振成像
影像特征
children
diffuse midline glioma with H3K27 altered
tomography
X-ray computer
magnetic resonance imaging
imaging features
