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酷似胶质母细胞瘤的中枢神经系统原发性血管炎:病例报告及文献综述

Primary Angiitis of the Central Nervous System Mimicking Glioblastoma: A Case Report and Literature Review.

作者信息

Jin Hang, Qu Yang, Guo Zhen-Ni, Cui Guo-Zhen, Zhang Fu-Liang, Yang Yi

机构信息

Department of Neurology, Stroke Center, The First Hospital of Jilin University, Changchun, China.

Department of Neurology, Clinical Trial and Research Center for Stroke, The First Hospital of Jilin University, Changchun, China.

出版信息

Front Neurol. 2019 Nov 13;10:1208. doi: 10.3389/fneur.2019.01208. eCollection 2019.

Abstract

Primary angiitis of the central nervous system (PACNS) is a rare disorder resulting in idiopathic inflammation affecting the parenchymal and leptomeningeal vessels confined to the central nervous system (CNS), of which a tumor-like mass lesion is an even rare subtype. We described a case of PACNS initially misdiagnosed as glioblastoma. The patient was a 35 year-old female with right-sided weakness and expressive dysphasia. Brain MRI showed a tumor-like lesion highly suggestive of glioblastoma, therefor surgical removal was done. After a resection and an exhaustive workup, PACNS was ultimately diagnosed. The case illustrates a type of imaging presentation of PACNS that is often misdiagnosed as high-grade glioma. Differentiation between tumor-like PACNS lesions and actual CNS tumors is challenging due to similar MR images. To avoid unnecessary surgical interventions, we summarized previously reported mass-forming PACNS cases in adults from January 1, 2000, to December 31, 2018 and the imaging characteristics of PACNS. Some less commonly used diagnostic methods such as MR spectroscopy may also help clinicians distinguish PACNS from its mimics.

摘要

中枢神经系统原发性血管炎(PACNS)是一种罕见的疾病,可导致特发性炎症,影响局限于中枢神经系统(CNS)的实质和软脑膜血管,其中肿瘤样肿块病变是一种更为罕见的亚型。我们描述了一例最初被误诊为胶质母细胞瘤的PACNS病例。该患者为一名35岁女性,有右侧肢体无力和表达性失语。脑部MRI显示一个高度提示胶质母细胞瘤的肿瘤样病变,因此进行了手术切除。经过切除和全面检查后,最终诊断为PACNS。该病例说明了PACNS的一种影像学表现类型,这种表现常被误诊为高级别胶质瘤。由于MR图像相似,区分肿瘤样PACNS病变与实际的中枢神经系统肿瘤具有挑战性。为避免不必要的手术干预,我们总结了2000年1月1日至2018年12月31日期间先前报道的成人肿块形成型PACNS病例以及PACNS的影像学特征。一些不太常用的诊断方法,如磁共振波谱,也可能有助于临床医生将PACNS与其相似疾病区分开来。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9e6b/6863980/21665b8b0ebf/fneur-10-01208-g0001.jpg

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