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高度恶性的非典型畸胎瘤样/横纹肌样瘤(atypical teratoid/rhabdoid tumor,AT/RT)极具侵袭性,为原发性中枢神经系统肿瘤。多发生在2岁以下的幼儿,且组织病理学和影像学与原始神经外胚层肿瘤(primitive neuroectodermal tumor,PNET)、髓母细胞瘤很相似。以往由于对此病认识不够,加之发病率很低,常常误诊。我们收集4例AT/RT,回顾性分析其MR平扫及增强表现,以提高临床对该病的认识。
Highly aggressive atypical teratoid / rhabdoid tumor (AT / RT) is a highly aggressive tumor of the primary central nervous system. Mostly occur in children under 2 years of age, and histopathology and imaging and primitive neuroectodermal tumor (PNET), medulloblastoma are very similar. In the past due to lack of awareness of the disease, combined with the low incidence, often misdiagnosed. We collected 4 cases of AT / RT, a retrospective analysis of its MR plain scan and enhanced performance, in order to improve clinical awareness of the disease.