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目的:探讨儿童中枢神经系统非典型畸胎样/横纹肌样瘤(AT/RT)的磁共振成像(MRI)表现。方法:回顾性分析本院2012年4月至2016年3月经手术病理证实的5例原发性AT/RT的MRI表现。结果:5名患儿平均年龄(26±1)个月。幕上1例,幕下4例,3例位于小脑,1例位于松果体区。肿瘤体积一般幕上大于幕下,平均直径约(4.9±1.4)cm,最大者为6.5 cm×6.8 cm×7.2 cm,位于右侧大脑半球。肿瘤一般为囊实性,信号混杂,T1WI等低信号为主者3例,T2WI呈等、高混杂信号5例,扩散加权成像上呈高信号者5例,边缘囊变5例,出血2例,增强后明显不均匀强化4例,轻度强化1例,实性部分呈环形条带样强化,瘤周轻度水肿2例。结论:AT/RT的磁共振影像表现具有一定特征,但无诊断特异性,结合患儿年龄和临床特点有助于该病的诊断。
Objective: To investigate the magnetic resonance imaging (MRI) of children with central nervous system atypical teratoid / rhabdomyosarcoma (AT / RT). Methods: MRI findings of 5 cases of primary AT / RT confirmed by surgery and pathology in our hospital from April 2012 to March 2016 were retrospectively analyzed. Results: The average age of five children (26 ± 1) months. 1 in the supratentorial site, 4 in the infratentorial site, 3 in the cerebellum, and 1 in the pineal region. Tumor volume is generally larger than the curtain on the screen, the average diameter of about (4.9 ± 1.4) cm, the largest of 6.5 cm × 6.8 cm × 7.2 cm, located in the right hemisphere. Tumors were generally cystic solidity, mixed signals, T1WI and other low signal mainly in 3 cases, T2WI was equal, high mixed signal in 5 cases, diffusion weighted imaging showed high signal in 5 cases, 5 cases of edge capsule change, bleeding in 2 cases , 4 cases were markedly uneven enhancement after enhancement, 1 case was mild enhancement, the solid part was annular band-like enhancement, 2 cases of mild peritumoral edema. CONCLUSIONS: The magnetic resonance imaging of AT / RT has certain characteristics but no diagnostic specificity. Combined with the age and clinical features of children, it is helpful to diagnose the disease.