Nasal Eosinophilic Angiocentric Fibrosis with Orbital Extension

Abstract

Eosinophilic angiocentric fibrosis (EAF) is an extremely rare, chronic, benign, idiopathic disorder that mostly affects the upper respiratory tract, particularly the nasal cavity, and features progressive submucosal perivascular fibrosis. To the best of our knowledge, only seven cases of EAF with orbital involvement have been reported. We report a case of sinonasal EAF with orbital extension that presented with left nasolacrimal duct obstruction. A 35-year-old man presented with left epiphora, proptosis, anterolateral globe displacement and nasal obstruction. Endoscopic sinus examination showed a firm, gritty, creamy, yellow, fibrous, adherent mass of maxillary sinus. Diagnosis was established with histopathological examination of excisional biopsy of the lesion. Although EAF is very rare, it should be considered in the differential diagnosis of lesions of upper airway tract, particularly the nasal cavity. Biopsy is necessary for diagnosis and treatment planning. Resecting of the involved tissues completely is essential for prevention of recurrence.

Diagnostic dilemmas of mechanical restriction of the medial rectus: A case report

“…18 Obliterative phlebitis is often absent in EAF, but eosinophilia is present within the lesion. 16–18 Although IgG4-RSD is known to involve the orbit, sinonasal tract, and, rarely, the EOM, 19–21 EAF and other IgG4-RSD are unlikely due to our patient’s unelevated serum IgG4 level, normal IgG4-to-IgG plasma cell ratio, and lack of both eosinophilic infiltrate and obliterative phlebitis on biopsy specimens.…”

A Case Report and Systematic Review of Eosinophilic Angiocentric Fibrosis of the Paranasal Sinuses

“…After eliminating duplicate cases (Rimmer et al and Paun described 4 of the same cases), there were a total of 58 case reports, plus the additional case from our institution, for a total of 59 cases. 1-2,4-11,13-41…”

Primary intraorbital inflammatory lumpy lesion: A rare case report

“…[17] Besides, there are scholars view that systemic and topical corticosteroids as ineffective. [18] But in any way, it is hard to diagnosis EAF without pathological results, and glucocorticoids appear to be effective initially. [19] Therefore, combined surgery to remove as much of the lesion as possible with glucocorticoid or other medicines like azathioprine, mycophenolate mofetil, and methotrexate as remission-maintenance drugs may be the best option at present.…”