Anti-neutrophil cytoplasmic antibody-associated central nervous system vasculitis mimicking brain tumor: A case report.

IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY Neuropathology Pub Date : 2024-10-30 DOI:10.1111/neup.13011
Yukiko Maeda, Ryotaro Ikeguchi, Kenta Masui, Atsushi Kurata, Kazuo Kitagawa, Yuko Shimizu
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Abstract

Here, we report a case of antineutrophil cytoplasmic antibody (ANCA)-associated central nervous system (CNS) vasculitis that mimicked a brain tumor. The patient presented with progressive right upper arm weakness. Brain magnetic resonance imaging (MRI) revealed large tumor-like lesions in the left frontal and parietal lobes, with patchy and irregular enhancement with gadolinium and edema. Based on the clinical course and radiological findings, a brain tumor was suspected, and stereotactic brain biopsy was performed. Brain histopathology revealed necrotic tissue and lymphocyte infiltration around small vessels and blood vessel walls. Although the patient's clinical course and pathological findings suggested primary angiitis of CNS (PACNS), double staining for myeloperoxidase (MPO) and CD31 (a neutrophil marker) revealed infiltration of MPO-positive neutrophils in the blood vessel walls. Therefore, we diagnosed the patient with ANCA-associated CNS vasculitis. Because CNS vasculitis, including PACNS, presents nonspecific clinical findings and can depict brain tumor-like MRI findings, CNS vasculitis should be carefully differentiated from brain tumors. Additionally, double staining for MPO and CD31 might be useful for evaluating the pathogenesis of CNS vasculitis.

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模仿脑肿瘤的抗中性粒细胞胞浆抗体相关中枢神经系统血管炎:病例报告。
在此,我们报告了一例与抗中性粒细胞胞浆抗体(ANCA)相关的中枢神经系统(CNS)血管炎病例,该病例模仿了脑肿瘤。患者出现进行性右上臂无力。脑磁共振成像(MRI)显示,患者左侧额叶和顶叶出现大面积肿瘤样病变,钆呈斑片状不规则强化,并伴有水肿。根据临床病程和放射学检查结果,怀疑是脑肿瘤,于是进行了立体定向脑活检。脑组织病理学检查显示,小血管和血管壁周围有坏死组织和淋巴细胞浸润。虽然患者的临床病程和病理结果均提示为中枢神经系统原发性血管炎(PACNS),但髓过氧化物酶(MPO)和 CD31(一种中性粒细胞标记物)双重染色显示血管壁有 MPO 阳性的中性粒细胞浸润。因此,我们诊断患者患有 ANCA 相关中枢神经系统血管炎。由于中枢神经系统血管炎(包括 PACNS)表现为非特异性临床表现,并可出现类似脑肿瘤的磁共振成像结果,因此中枢神经系统血管炎应与脑肿瘤仔细鉴别。此外,MPO 和 CD31 的双重染色可能有助于评估中枢神经系统血管炎的发病机制。
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来源期刊
Neuropathology
Neuropathology 医学-病理学
CiteScore
4.10
自引率
4.30%
发文量
105
审稿时长
6-12 weeks
期刊介绍: Neuropathology is an international journal sponsored by the Japanese Society of Neuropathology and publishes peer-reviewed original papers dealing with all aspects of human and experimental neuropathology and related fields of research. The Journal aims to promote the international exchange of results and encourages authors from all countries to submit papers in the following categories: Original Articles, Case Reports, Short Communications, Occasional Reviews, Editorials and Letters to the Editor. All articles are peer-reviewed by at least two researchers expert in the field of the submitted paper.
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