Clinical Case Analysis of Atypical Malformation/Rhabdoid Tumor of the Central Nervous System
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摘要:
目的:探讨中枢神经系统非典型畸胎样/横纹肌样瘤(AT/RT)的临床表现、病理特征及影像学特点,以提高对本病的影像诊断及鉴别诊断的能力。方法:回顾分析1例经手术后病理证实为中枢神经系统非典型畸胎样/横纹肌样瘤的临床表现、影像表现,并复习相关文献。结果:CT平扫示右额部稍高密度团块状病灶,占位效应明显,周围见结片状水肿带,骨窗显示邻近骨质无破坏;MRI显示病灶在T1WI呈低信号,T2WI及液体衰减反转恢复(FLAIR)呈稍高信号,扩散加权成像(DWI)序列呈高信号,病灶信号较均匀,呈分叶状,MRI增强示病灶欠均匀强化,部分条絮样明显强化,病灶以宽基底与大脑镰相连。结论:中枢神经系统非典型畸胎样/横纹肌样瘤罕见,临床表现变现多样化,影像表现缺乏特异性,确诊仍然需要病理组织学检查。
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关键词:
- CT/MRI诊断 /
- 中枢神经系统 /
- 非典型畸胎样/横纹肌样瘤
Abstract:Objective: To explore the clinical manifestations, histopathological features, and imaging characteristics of atypical malformation/rhabdoid tumor (atypical teratoid/rhabdoid tumor) of the central nervous system, to improve the ability of imaging diagnosis and differential diagnosis of this disease. Methods: Clinical realization and imaging findings of an atypical abnormal pattern/rhabdoid were reviewed. Results: Computed tomography revealed a slightly dense right frontal lobe and the lesion had a significant occupancy effect with a visible band of nodular edema around it. The bone window showed no adjacent bone destruction. Magnetic resonance imaging (MRI) revealed the lesion as low signal on T1 and T2-weighted imaging, and fluid attenuation and inversion recovery had a slightly higher signal. A diffusion-weighted imaging (DWI) sequence showed high signal, uniform lesion signal, and lobulation. MRI enhancement showed less uniform enhancement, edge line enhancement, and a broad base attached to the brain stem. Results: Atypical teratoid/rhabdoid tumors of the central nervous system are rare, with diverse clinical manifestations, lack of specificity of imaging findings, and histopathological examination is still required for diagnosis.
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