Unveiling primary intracranial eosinophilic angiocentric fibrosis: A rare case report and diagnostic dilemmas.

IF 1.3 4区 医学 Q4 CLINICAL NEUROLOGY
Neuropathology Pub Date : 2024-08-06 DOI:10.1111/neup.12997
Rumela Nayak, Bevinahalli N Nandeesh
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引用次数: 0

Abstract

Eosinophilic angiocentric fibrosis (EAF) is a rare, benign fibroinflammatory condition primarily affecting the sinonasal and upper respiratory tract, with a few cases reported beyond these regions. Primary intracranial EAF is rare. To date, only one case of intracranial EAF has been reported; ours is the second. This case report presents a case of EAF in a 55-year-old man, initially misdiagnosed as meningioma based on clinical and radiological features. The patient complained of a persistent dull headache for six months without associated neurological symptoms. Brain magnetic resonance imaging revealed a dural-based lesion with characteristics suggestive of meningioma. However, histopathological examination post-surgical resection revealed a nodular vascular lesion with concentric angiocentric fibrosis, a distinctive onion skin pattern, and an inflammatory infiltrate rich in eosinophils, plasma cells, and histiocytes. Immunohistochemistry ruled out IgG4-related disease, and other systemic disorders were ruled out based on combined clinical and histological features. This case underscores the need for considering EAF in the differential diagnosis of dural-based lesions. Awareness of its potential mimicking of meningioma is crucial for accurate diagnosis and appropriate management, emphasizing the importance of histopathological examination in challenging cases.

揭开原发性颅内嗜酸性粒细胞血管中心纤维化的神秘面纱:罕见病例报告与诊断难题。
嗜酸性粒细胞血管中心纤维化(EAF)是一种罕见的良性纤维炎症,主要累及鼻窦和上呼吸道,少数病例报告超出了这些区域。原发性颅内 EAF 很少见。迄今为止,仅有一例颅内 EAF 的报道,我们是第二例。本病例报告介绍了一例颅内 EAF 病例,患者是一名 55 岁的男性,最初根据临床和放射学特征被误诊为脑膜瘤。患者主诉持续钝性头痛 6 个月,无相关神经症状。脑磁共振成像显示,硬脑膜病变具有脑膜瘤的特征。然而,手术切除后的组织病理学检查显示,病变为结节状血管病变,伴有同心圆状血管中心纤维化、独特的洋葱皮形态以及富含嗜酸性粒细胞、浆细胞和组织细胞的炎性浸润。免疫组化检查排除了 IgG4 相关疾病,根据临床和组织学综合特征排除了其他系统性疾病。该病例强调了在硬脑膜病变的鉴别诊断中考虑 EAF 的必要性。认识到EAF可能与脑膜瘤相似是准确诊断和适当处理的关键,强调了组织病理学检查在疑难病例中的重要性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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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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