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目的:探讨中枢神经系统非典型畸胎瘤样/横纹肌样瘤(atypical teratoid/rhabdoid tumors，AT/RT)的临床与神经影像学特点及不同位置的生存预后。方法:收集2012年10月至2020年9月首都医科大学三博脑科医院共计27例经活检或术后病理证实的AT/RT患者的临床资料，其中男17例，女10例；年龄(7.6±10.0)岁，年龄范围为0.2～39.0岁。对其临床特征及首次术前影像学检查结果进行回顾性总结分析。并根据发生位置分为幕上、幕下和跨幕上幕下组3组，应用Kaplan-Meier生存曲线比较3组患者的生存时间。结果:本组患者均表现为头痛，其中12例伴呕吐。位于幕上12例、幕下10例、跨幕上幕下5例。含囊变20例，钙化7例，出血6例，瘤周水肿13例。幕下AT/RT患者较幕上及跨幕上幕下中位生存时间长，差异有统计学意义(χn 2＝7.353，n P＝0.025)。n 结论:中枢神经系统AT/RT易发生于低龄儿童，发生于幕下的AT/RT患者生存时间较长。“,”Objective:To investigate the clinical and neuroimaging features of atypical teratoid/rhabdoid tumors (AT/RT) of the central nervous system and the survival prognosis in different locations.Methods:The clinical data of 27 patients with AT/RT confirmed by biopsy or postoperative pathology in Sanbo Brain Hospital of Capital Medical University from October 2012 to September 2020 were collected, including 17 males and 10 females, aged (7.6±10.0) years, ranging from 0.2 to 39.0 years old.The clinical features and the results of the first preoperative imaging examination were retrospectively analyzed.The patients were divided into supratentorial, infratentorial and trans supratentorial and infratentorial groups according to the location.The survival time of the three groups was compared by Kaplan-Meier survival curve.Results:All patients presented with headache, including 12 cases with vomiting.There were 12 cases of supratentorial, 10 cases of infratentorial and 5 cases of supratentorial and infratentorial.There were 20 cases of cystic degeneration, 7 cases of calcification, 6 cases of hemorrhage and 13 cases of peritumoral edema.The median survival time of patients with infratentorial AT/RT was longer than that of patients with supratentorial and transtentorial AT/RT (χn 2=7.353, n P=0.025).n Conclusion:Central nervous system AT/RT is easy to occur in young children, and the survival time of AT/RT patients is longer.