颅内非典型畸胎瘤样／横纹肌样瘤的影像学表现（附4例报道和文献复习）

Imaging Findings in Intracranial Atypical Teratoid/Rhabdoid Tumors：Report of 4 Cases and Litera-ture Review

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，提高对其认识水平。方法：收集我科1997年1月～2011年12月经病理和免疫组化诊断为AT/RT的4例患者的临床资料，对其影像学表现进行回顾性分析，并进行相关文献复习。结果：4例中，病灶位于额叶2例，颞叶1例，桥脑小脑角区（CPA）1例；最大径3．6～6．5 cm。肿瘤边界清楚，3例轻度瘤周水肿，1例无瘤周水肿；4例轻度占位效应，位于CPA的1例可见同侧内听道扩大。肿瘤实质在CT上呈等密度，MRI T1WI呈低信号、T2 WI呈高低混杂信号。4例肿瘤内均见出血，2例见囊变。增强后肿瘤实质呈斑片状强化，囊壁和分隔亦见强化。结论：AT/RT的影像学表现有一定的特征性，但缺乏特异性；提高对本病的认识是诊断的关键。

Objective：To investigate the imaging features of intracranial atypical teratoid /rhab doid tumors （ AT/RT） , and to improve the understanding of it .Methods：Clinical data of four cases with AT/RT proved by pathology and immunohistochemical were collected from Jan .1997 to Dec.2011.The imaging appear-ance was retrospectively analyzed , and related articles were reviewed .Results：Two cases were located in frontal lobe, one case in temporal lobe and one case in cerebellopontine angle （ CPA）.The maximum diameter of tumors was 3.6~6.5 cm.The border of tumors was well -defined.Peritumor edema was mild in three lesions and none in one lesion .All of tumors had mild space occupying effect , and internal auditory canal enlarged in the CPA lesion.The solid portions of lesions were intermediate attenuation on CT , low signal on T1WI, hyper-and hypo-intensity on T2WI.All of tumors had hemorrhage, and two cases had necrotic cystic change .Af-ter contrast media injection , the solid part of tumor showed patch enhancement , with cystic wall and separation enhancement .Conclusions：The imaging appearance of AT/RT has some characteristics , but not specific .Im-provement the recognition of this disease is the key to correct diagnosis .