Eosinophilic Angiocentric Fibrosis: An Uncommon Entity in the Spectrum of IgG4-related Disease.

Head and neck pathology Pub Date : 2025-05-26 DOI:10.1007/s12105-025-01795-x

Sarah E Gradecki, Edward B Stelow

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Abstract

Background: Eosinophilic angiocentric fibrosis (EAF) is a rare, tumefactive inflammatory process of the sinonasal and upper respiratory tract and orbit. Numerous histologic mimickers of EAF occur in these locations.

Methods: A comprehensive literature review focused on EAF and its histologic mimickers was performed.

Results: This manuscript serves as an overview of the histopathological characteristics of EAF and its histologic mimickers, including granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), sinonasal inflammatory polyps, epithelioid hemangioma, invasive fungal rhinosinusitis, Langerhans cell histiocytosis, rhinoscleroma, inflammatory myofibroblastic tumor, and fibromatosis.

Conclusion: A correct diagnosis of EAF can be reached by identifying the classic histopathologic features of prominent eosinophilia, lymphoplasmacytic inflammation, and concentric perivascular fibrosis. Additionally, as EAF sometimes lies on the spectrum of IgG4-related disease, immunohistochemistry and serologic studies can be used to aid in diagnosis.

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嗜酸性粒细胞血管中心性纤维化：igg4相关疾病谱中的罕见实体

背景：嗜酸性血管中心性纤维化（EAF）是一种罕见的鼻窦、上呼吸道和眼眶的肿瘤性炎症过程。许多EAF的组织学模拟物发生在这些部位。方法：对EAF及其组织学模拟物进行全面的文献回顾。结果：本文综述了EAF及其组织模拟物的组织病理学特征，包括肉芽肿伴多血管炎（GPA）、嗜酸性肉芽肿伴多血管炎（EGPA）、鼻窦炎性息肉、上皮样血管瘤、侵袭性真菌性鼻窦炎、朗格汉斯细胞组织细胞增生症、鼻硬化瘤、炎性肌纤维母细胞瘤和纤维瘤病。结论：EAF的典型病理表现为明显的嗜酸性粒细胞增多、淋巴浆细胞性炎症和同心性血管周围纤维化，可得到正确的诊断。此外，由于EAF有时位于igg4相关疾病的频谱上，免疫组织化学和血清学研究可用于帮助诊断。

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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Head and neck pathology

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