Nasal fibrosis: long-term follow up of four cases of eosinophilic angiocentric fibrosis

Abstract

Eosinophilic angiocentric fibrosis is a rare, benign cause of submucosal thickening and fibrosis within the upper respiratory tract. It predominantly affects the nose although cases have been reported in the subglottis. We describe four cases of the disease centred around the nasal cavity, with widespread infiltration of the facial soft tissues and orbit in three of the four patients. Each underwent long term follow up. Multiple surgical resections were required with two of our patients and, to date, medical therapy has been of limited help. The disease process, with its clinical and characteristic histopathological findings, is described. We also discuss the management of the disease following a comprehensive review of, and comparison with, the few prior reported cases.

Rare Pathology of Sinonasal Tract; Eosinophilic Angiocentric Fibrosis

“…Allergic etiology is proposed to be responsible due to predominance of eosinophil rich inflam-mation (2,3,7,8). However, only some cases have a history of allergy in the literature.…”

“…However, only some cases have a history of allergy in the literature. Also, EAF has been found to be refractory to steroids and the other anti-allergic therapies (7,8). Trauma was also thought to be a predisposing factor but this was not supported by cases in the literature (2).…”

Sinonasal Eosinophilic Angiocentric Fibrosis: A Report of Four Cases and Review of Literature

“…The recurrence rate is extremely high, with persistence of disease seen following most nasal resections [2-4, 6, 7, 9, 11]. Other treatment modalities, including local and systemic corticosteroid therapy have been tried with minimal clinical resolution of disease [2,6,7,17,18,25]. No definitive treatment of choice has been recognized, and the etiology of EAF remains elusive despite the consistency of pathological findings in the described EAF cases.…”

“…Surgical resection is the treatment of choice, though multiple procedures are often required. Though a possible association between GF and EAF, and WG and EAF have been reported, further case reports of this rare lesion are necessary before the etiology, pathogenesis and management can be clearly defined [1,2,7,8,12,[14][15][16][17][18]23].…”

“…Two years later, Roberts and McCann reported two cases of female patients with an unusual stenosing lesion involving the upper respiratory tract and gave a descriptive diagnosis: eosinophilic angiocenteric fibrosis (EAF) [2]. A review of the English literature reveals 28 additional cases of EAF involving the sinonasal tract have been reported [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22]. EAF typically presents in young to middle-aged females as a slowly progressive upper airway obstruction in association with a submucosal inflammatory, fibrosing tumor-like lesion.…”