颅内非典型畸胎瘤样/横纹肌样瘤的影像学表现(附6例报道)  

Imaging findings of intracranial atypical teratoid/rhabdoid tumors:report of 6 cases and literature review

作　　者：沈文婷[1] 沈全力[1] 乔中伟[1] 

机构地区：[1]复旦大学附属儿科医院放射科,上海201102

出　　处：《放射学实践》2015年第6期638-642,共5页Radiologic Practice

摘　　要：目的：探讨颅内非典型畸胎瘤样/横纹肌样瘤（AT/RT）的影像学特征。方法：回顾性分析2010年10月-2014年6月经病理和免疫组化诊断为AT/RT的6例患者的CT（n=3）和MRI（n=6）表现,并进行相关文献复习。结果：6名患者平均年龄2.05岁。肿瘤位于幕上4例,幕下1例,幕上及幕下多发病灶1例。肿瘤最大径30～96mm,平均（59±24）mm。囊变6例,出血4例,钙化2例,所有病灶都有中重度强化。病灶扩散加权成像（DWI）呈不均匀高信号,表观扩散系数（ADC）值（0.70±0.12）×10^-3 mm^2/s。1例病灶侵犯邻近硬膜及颅骨;1例病灶发生柔脑膜种植。2例患者术后随访MRI时病灶复发。结论：虽然颅内AT/RT无特异性影像学表现,但当肿瘤较大、伴有囊变及出血、低ADC值及脑脊液播散时,不应排除AT/RT的诊断。Objective：To explore the imaging features of intracranial atypical teratoid/rhabdoid tumors（AT/RTs）.Methods：6patients with AT/RTs proved by pathology and immunohistochemical test were collected from Oct 2010 to Jun2014.The pre-operative CT（n=3）and MRI（n=6）features were retrospectively characterized,with a review of the literature from PubMed database.Results：The mean age of patients was 2.05 years.The neoplasms were supratentorial in 4cases,infratentorial in 1case and multifocal（involving in both infra and supratentorial regions）in 1case.The maximum diameter of tumors was varying from 30 to 96mm;mean diameter was（59±24）mm.All AT/RTs showed marked heterogeneity on both CT and MRI images secondary to cystic changes in 6cases,hemorrhage in 4cases and calcification in 2cases.All cases showed moderate（n=1）to severe（n=5）enhancement.Leptomeningeal dissemination（n=1）and invasion of the adjacent skull（n=1）were found by MRI at the diagnosis.At clinical follow-up,the tumor recurrence was found by MRI in 2cases 1month and 8months after the surgery.Diffused-weighted imaging demonstrated inhomogeneous hyperintense signal for all 6patients,with a mean ADC value of（0.70±0.12）×10^-3 mm^2/s.Conclusions：Bulky,heterogeneous mass with cysts,hemorrhage,low ADC value and cerebral spinal fluid dissemination in pediatrics should alert the radiologist to take AT/RTs into the diagnosis consideration.

关 键 词：非典型畸胎瘤样/横纹肌样瘤 磁共振成像 诊断 

分 类 号：R739.41[医药卫生—肿瘤] R730.44[医药卫生—临床医学] R445.2