颅内非典型畸胎瘤样/横纹肌样瘤一例

目的探讨颅内非典型畸胎瘤样/横纹肌样瘤(AT/RT)的临床表现、影像学、组织病理学特点及其治疗和预后。方法报告一例颅内非典型畸胎瘤样/横纹肌样瘤病例临床资料,结合文献进行分析。结果颅内AT/RT是中枢神经系统罕见的高度恶性肿瘤,临床及影像学表现缺乏特异性,病理组织学改变是确诊的主要依据。结论 AT/RT是好发于儿童的颅内罕见恶性肿瘤,预后极差。免疫组化染色对确诊AT/RT十分重要。

A case report of intracranial atypical teratoid/rhabdoid tumor

Objective To study the clinical manifestation, imageological and histopathological features of intracranial atypical teratoid/rhabdoid tumor, and finally discussed the treatment and prognosis. Methods One case of intracranial atypical teratoid/rhabdoid tumor was presented and discussed combinding with the detailed literature analysis. Results Intracranial atypical teratoid/rhabdoid tumors were rare and extremely mahgnant tumors in the central nervous system, it.s distinct clinical manifestation and neuroradiologic features were poorly characterized, and its＇ diagnosis mainly depended on the histopathologic characterize. Conclusions Atypical teratoid/rhabdoid tumors are rare malignant neoplasms occurring mainly in the early childhood, their prognosis are worst. The significance of immunohistochemical studies for making a correct dignosis are emphasized.