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揭示原发性颅内嗜酸性血管中心性纤维化为罕见病例报告及诊断困境。

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

机构信息

Department of Neuropathology, National Institute of Mental Health and Neurosciences, Bengaluru, India.

出版信息

Neuropathology. 2024 Dec;44(6):459-463. doi: 10.1111/neup.12997. Epub 2024 Aug 6.

DOI:10.1111/neup.12997
PMID:39105360
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.

摘要

嗜酸性血管中心性纤维化为一种罕见的良性纤维炎性病变,主要影响鼻旁窦和上呼吸道,也有少数病例发生于这些部位以外。颅内原发性嗜酸性血管中心性纤维化罕见。迄今为止,颅内嗜酸性血管中心性纤维化仅报告过一例,我们报告的是第二例。本文报告了一例 55 岁男性颅内嗜酸性血管中心性纤维化病例,最初根据临床和影像学特征误诊为脑膜瘤。患者诉持续钝痛性头痛 6 个月,无相关神经系统症状。脑部磁共振成像显示一硬脑膜为基底的病变,具有脑膜瘤的特征。然而,术后切除的组织病理学检查显示结节状血管病变,具有同心性血管中心性纤维化、特征性洋葱皮样模式和富含嗜酸性粒细胞、浆细胞和组织细胞的炎症浸润。免疫组化排除了 IgG4 相关疾病,根据临床和组织学特征综合排除了其他系统性疾病。该病例强调了在硬脑膜为基底病变的鉴别诊断中需要考虑嗜酸性血管中心性纤维化。认识到其可能类似于脑膜瘤对于准确诊断和适当管理至关重要,强调了在具有挑战性的病例中组织病理学检查的重要性。

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Unveiling primary intracranial eosinophilic angiocentric fibrosis: A rare case report and diagnostic dilemmas.揭示原发性颅内嗜酸性血管中心性纤维化为罕见病例报告及诊断困境。
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