Extranasopharyngeal angiofibroma: etiology, incidence and management

Angiofibromas in the head and neck area usually arise in the nasopharynx (NA) in adolescent males. They may also occur outside the nasopharynx (ENA) and can therefore be misdiagnosed. An ill-advised biopsy may result in brisk bleeding. This study was undertaken to evaluate the incidence and clinical features of ENA.

A review of the international literature was performed.

A total of 65 patients with ENAs from 16 different countries were reported in the literature. Two patients had a congenital lesion, the oldest being 78 years old. A total of 48 patients were male (73%). The maxilla was the most commonly affected site (24.6%), with the ethmoid, nasal cavity or septum and other sites being involved less frequently. Symptoms arose in 40 patients within 6 months. Among a wide variety of symptoms, epistaxis with or without nasal obstruction was reported for 18 patients. Brisk bleeding resulting from a total of 23 biopsies occurred in 13 patients, and required blood transfusion in 11. Death was reported for two patients as a result of acute respiratory compromise and endocranial extension.

ENAs are extremely rare compared to NAs and have to be acknowledged as a different entity. In comparison to patients with NAs, as female adults are affected, the lesion is diagnosed earlier and is less vascularized and the patients are older. Surgical resection is sufficient treatment due to a tendency for local and less aggressive growth.