Eosinophilic angiocentric fibrosis of the sinonasal tract: a case report and review of the literature

Abstract

Eosinophilic angiocentric fibrosis (EAF) is a rare chronic benign disorder of unknown etiology and is characterized by submucosal thickening and fibrosis in the upper respiratory tract. In this report, we describe a case of EAF in the nasal cavity of a woman who underwent elective surgery for division of adhesions and has had no recurrence during 2 years of postoperative follow-up. A review of the literature on the clinical manifestations of EAF, sites of lesions, management, and outcomes identified 48 articles that included 72 cases. A summary of these reports is presented, including our present case. The most common anatomic site involved was the nose (77.8%), the most common manifestation was nasal obstruction (66.7%), and the most common treatment modality was surgical resection (83.3%). After surgery, 36% of patients remained free of EAF. The most common pharmacologic agent used was a corticosteroid (38.9%).

Primary intraorbital inflammatory lumpy lesion: A rare case report

“…The characteristic histologic feature of EAF are as follows: a dense fibrotic stroma with a perivascular “onion skin-like” whorling pattern and a dense inflammatory infiltrate that the major cells are eosinophils, consisting of lymphocytes, plasma cells, and some neutrophils. [11] Microscopic pathology in our case showed proliferative fibrous tissue, eosinophils, lymphocytes and plasma cells. And they distributed in concentric circles around the vessel.…”

A rare lumpy inflammatory lesion of the orbit: a unique case report

“…Nasal congestion, nasal bleeding, loss of smell and other symptoms can also be associated with naso-orbital communication lesions. Studies have shown that nasal congestion is the most common symptom of nasal EAF, appearing in about 66.7% of cases [ 1 ]. Six cases had extraocular-muscle involvement, mostly of the internal rectus muscle.…”

“…Histopathological examination is the main standard for diagnosing EAF. The typical pathological manifestations are massive infiltration of eosinophils, plasma cells, lymphocytes, and fibroblasts around small blood vessels; thickening of matrix; and characteristic rotation of onionskin-like collagen fibers and mesh proteins around occlusive blood vessels, accompanied by positive expression of IgG4 and IgG [ 1 , 2 ]. EAF should be distinguished from orbital inflammatory pseudotumor, hemangioma, and granulomatous disease.…”

“…Eosinophilic angiocentric fibrosis (EAF) is a rare and slow progressive inflammatory disease. The main sites of involvement are the nasal cavity and sinuses, although rare cases can also occur in the orbit, upper respiratory tract, gingiva, and brain [ 1 – 4 ]. The etiology and pathogenesis of this disease are still unclear, and its clinical manifestations are nonspecific and diverse, making it easy to confuse with orbital inflammatory lesions or neoplastic lesions in diagnosis.…”