Original Contribution
Eosinophilic angiocentric fibrosis of the sinonasal tract

https://doi.org/10.1016/j.anndiagpath.2007.02.002Get rights and content

Abstract

Eosinophilic angiocentric fibrosis (EAF) is an uncommon inflammatory fibrosing lesion of the upper respiratory tract and orbit that occurs mainly in young to middle-aged women. The etiology of EAF is unknown. To our knowledge, approximately 28 cases have been previously reported in the English literature. We report here 3 additional cases of EAF of the sinonasal tract; 2 in women aged 19 and 31 years, and 1 in a man aged 49 years. The 19-year-old woman is the youngest patient with EAF ever described. The patients presented with a nasal cavity mass, face pain, or nasal obstructive symptoms of long duration.

Introduction

Eosinophilic angiocentric fibrosis (EAF) is a rare, benign, obstructive lesion affecting the respiratory mucosa of the nasal cavity, larynx, and orbit. Since its first description in 1985 by Roberts and McCann [1], approximately 28 cases of EAF have been described in the English literature. The specific histologic features include a perivascular inflammatory cell infiltration (mainly eosinophils), which is gradually replaced with progressive fibrosis (“onion-skin” pattern) around small blood vessels [1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11], [12], [13], [14], [15], [16], [17]. The etiology of EAF is unknown, and diagnosis is based on specific histologic findings. We report 3 cases of EAF, including one of a 19-year-old woman, the youngest patient described so far with this disease.

Section snippets

Case reports

The pertinent clinical history, therapy, and follow-up of the 3 patients with EAF of the sinonasal tract are summarized in Table 1.

Pathologic findings

The histologic appearance of the EAF was almost identical for all 3 cases. The normal architecture of the maxillary sinus (case 1), the right side of the nasal cavity (case 2), and the nasal septum (case 3) were completely distorted by fibrous-collagenous proliferation with a variable dense inflammatory infiltrate. The fibrous component displayed a concentrically layered onion-skin–type perivascular arrangement (Fig. 1A, B, and C), which is characteristic of EAF. The inflammatory infiltrate

Treatment and follow-up

The lesions were completely curettaged, and the patients were free of disease at 1, 7, and 12 years, respectively (see Table 1).

Discussion

Although most of the 28 reported cases of EAF occurred in the nasal cavity (25 cases), including 3 cases with extension into the orbit, 2 cases occurred in the larynx, and 1 case occurred in the orbit (Table 1, Table 2). Patient ages ranged from 25 to 79 years (median, 50.5 years), with a slight female preponderance (female-male ratio, 1.5/1).

Eosinophilic angiocentric fibrosis is a slowly progressing disease that can exist for more than 4 years before it is diagnosed [1], [2], [3], [6]. The

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