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Sinonasal tract eosinophilic angiocentric fibrosis. A report of three cases
1Department of Endocrine and Otorhinolaryngic-Head & Neck Pathology, Armed Forces Institute of Pathology, 6825 16th St, NW, Washington, DC 20306-6000, USA.
Abstract:
Eosinophilic angiocentric fibrosis (EAF) is a rare submucosal fibrosis without a well-developed differential diagnosis. Three cases of sinonasal tract EAF were identified in 2 women and 1 man, aged 49, 64, and 28 years, respectively. The patients experienced a nasal cavity mass, maxillary pain, or nasal obstructive symptoms of long duration. The process involved the nasal septum (n = 2), nasal cavity (n = 1), and/or the maxillary sinus (n = 1). There was no evidence for Wegener granulomatosis, Churg-Strauss syndrome, Kimura disease, granuloma faciale, or erythema elevatum diutinum. Histologically, the lesions demonstrated a characteristic perivascular "onion-skin" fibrosis and a full spectrum of inflammatory cells, although eosinophils predominated. Necrosis and foreign body-type giant cells were not identified. Surgical excision was used for all patients, who are all alive but with disease at last follow-up. Sinonasal tract EAF is a unique fibroproliferative disorder that does not seem to have systemic associations with known diseases. The characteristic histomorphologic features permit accurate diagnosis.
Insights
Eosinophilic angiocentric fibrosis (EAF) is a rare sinonasal disorder. Its distinct histopathology aids diagnosis, differentiating it from other conditions.
Area of Science:
- Pathology
- Otolaryngology
Background:
- Eosinophilic angiocentric fibrosis (EAF) is a rare submucosal fibrotic condition.
- A well-defined differential diagnosis for EAF is lacking.
Observation:
- Three cases of sinonasal EAF were analyzed in two women and one man.
- Patients presented with chronic symptoms including nasal masses, maxillary pain, and nasal obstruction.
- Lesions involved the nasal septum, nasal cavity, and maxillary sinus.
Findings:
- Histological examination revealed characteristic perivascular "onion-skin" fibrosis with predominant eosinophils.
- Inflammatory cells were present, but necrosis and foreign body giant cells were absent.
- Differential diagnoses, including Wegener granulomatosis and Churg-Strauss syndrome, were excluded.
Implications:
- Sinonasal EAF is a distinct fibroproliferative disorder without apparent systemic associations.
- Characteristic histomorphologic features are crucial for accurate diagnosis.
- Surgical excision is a primary treatment modality, though disease may persist post-operatively.
