Original ContributionEosinophilic angiocentric fibrosis of the sinonasal tract
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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2023, Annals of Diagnostic PathologyCitation Excerpt :Following its original description by Holmes and Panje in 1983 as intranasal granuloma faciale [2], the currently used term “eosinophilic angiocentric fibrosis (EAF)”, has been coined by Roberts and McCann in 1985 [3]. To date, no >60 cases have been reported in the literature [1,4-7]. EAF affects both sexes equally at a wide age range (16 to 79 yrs).
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2010, Diagnostic HistopathologyCitation Excerpt :In later stages the inflammatory infiltrate is more patchy and mixed (eosinophils, neutrophils, lymphocytes and plasma cells) and progresses to onion-skin perivascular fibrosis (Figure 21). The clinical and imaging features are non-specific and the diagnosis is histological.44 The differential diagnosis includes most of the other chronic inflammatory conditions mentioned in this review.
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