Abstract

Nasopharyngeal angiofibroma (NA) is a rare, vascular tumor affecting adolescent males. Due to aggressive local growth, skull base location and risk of profound hemorrhage, NA is a challenge for surgeons. Angiofibromas have been sporadically described in extanasopharyngeal locations. We review ten cases of extranasopharyngeal angiofibroma (ENA) and discuss the incidence, clinical presentation and management of this pathology. The group consisted of 4 males and 5 females aged 8–49. There were 7 patients with nasal angiofibroma, 1 patient with laryngeal angiofibroma, 1 patient with oral angiofibroma and another patient with infratemporal fossa tumor. In patients with nasal angiofibroma most common presenting symptoms were nasal obstruction and epistaxis. Patients with laryngeal angiofibroma suffered from mild dysphagia and patients with the infratemporal fossa tumor had painless cheek swelling. In four patients with nasal tumor computed tomography (CT) demonstrated mass with strong to intermediate contrast enhancement. In one patient with nasal tumor carotid angiography demonstrated pathological vessels without intensive tumor blush. Infratemporal fossa tumor showed intensive contrast enhancement on CT and magnetic resonance imaging (MRI) scans, and abundant vascularity on angiography. Laryngeal and oral angiofibroma required no radiological imaging. Three nasal tumors were evaluated before introduction of CT to clinical practice. All patients underwent surgery. No recurrences developed. ENAs differ significantly from NAs regarding clinical and radiological presentations. They lack typical clinical and radiological features as they develop in all age groups and in females, may be less vascularised, arise from various sites and produce a variety of symptoms.

Highlights

  • Nasopharyngeal angiofibroma (NA) is a rare vascular tumor, which represents 0.05 % of all head and neck tumors

  • We review ten cases of extranasopharyngeal angiofibroma (ENA) and discuss the incidence, clinical presentation and management of this pathology

  • We present ten cases of extranasopharyngeal angiofibroma arising from various sites and discuss the incidence, clinical presentation and management of this pathology in comparison to NAs

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Summary

Background

Nasopharyngeal angiofibroma (NA) is a rare vascular tumor, which represents 0.05 % of all head and neck tumors. Histologically benign it shows locally aggressive growth with bone destruction and spread through natural foramina and fissures. It originates from the posterolateral wall of the nasopharynx and from this site usually extends to the nasopharynx, nasal cavity, paranasal sinuses, spheno-palatine foramen and infratemporal fossa. In 10–20 % of the cases tumor invades the cranial cavity [2]. Extranasopharyngeal angiofibromas (ENAs) have been sporadically described in the literature. We present ten cases of extranasopharyngeal angiofibroma arising from various sites and discuss the incidence, clinical presentation and management of this pathology in comparison to NAs

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