Extranasopharyngeal angiofibroma revisited

Abstract

Background Angiofibromas in the head and neck region usually arise in the nasopharynx, but may also occur elsewhere. This study aims at evaluating the incidence and clinical features of extranasopharyngeal angiofibroma (ENA). Material and Methods Systematic review of the literature (Medline® and Google™) up to 31 December 2015. Results 174 cases of ENA were retrieved from a total of 170 publications. In contrast to former publications and previous understanding, the nasal septum was by far the most common site of the disease. Four patients had a congenital lesion, the oldest patient was 87 years old (mean: 28.7 years; median: 23 years). Male gender was predominantly affected, but the sex ratio was more balanced (2.13:1) than in previous reports in the literature until 12/2015. The majority of patients presented with nasal obstruction, either in combination with epistaxis (25.8%) or other symptoms (12.6%). Symptoms had developed within 13.1 months on average (median: 4 months). Brisk bleeding resulted in 11 of 43 biopsy procedures. Surgical resection as first‐line therapy was performed in 170 patients. A tumour regrowth within 12 months was registered in four patients. Conclusion The increasing awareness of ENA and the willingness to publish case reports—not only in Medline‐listed journals—resulted in a significant increase of published case reports lately. Although extremely rare, ENAs have to be taken into account in the differential diagnosis of unclear masses, particularly in adult patients presenting with a rapidly developing nasal obstruction resulting from a nasal septum tumour. Female gender or normal vascularity does not exclude the diagnosis. Transnasal resection is sufficient in most cases, and recurrences are rare. Pathologists as well as clinicians should consider ENA in their differential diagnosis of any mass of the upper airway.

Uncommon presentations of angiofibroma in sinonasal tract: a two-case report/ Apresentações incomuns de angiofibromas no trato nasossinusal: relato de dois casos

“…The most common site of ENPA reported in literature is the maxillary sinus 4 , although angiofibromas can occur anywhere in the mucosa of the upper respiratory tract. The clinical presentation of ENPA can be extremely variable however it often has epistaxis as primary symptoms 5 . The etiology of the ENPA is still unclear.…”

Frontoethmoidal Extranasopharyngeal Angiofibroma With Orbital Pyocele

“…Unlike JNA, ENA shows a low recurrence rate of 2.3%. 3 Differential diagnosis for a vascular frontoethmoid mass in an adult could include tumors such as hemangiopericytoma, hemangioma, and angiosarcoma and ENA is a rare possibility. 12 Awareness of this rare entity will help in better preoperative assessment ensuring complete surgical excision.…”

Extranasopharyngeal Angiofibroma Arising from the Anterior Nasal Septum in a 35-Year-Old Woman

“…Angiofibroma is a histologically benign but locally aggressive, nonencapsulated, highly vascular tumor, usually originating in nasopharynx and affecting mostly male adolescents 1. The most common form of angiofibroma with primary nasopharyngeal localization is termed “juvenile nasopharyngeal angiofibroma (JNA).” According to recent investigations, JNA does not originate in the nasopharynx, as previously assumed, but rather from a fibrovascular nidus in the posterior part of the nasal cavity, near by the sphenopalatine foramen, representing approximately 0.05% of all tumors of the head and neck 2.…”

“…The incidence in other age group and in women is extremely low. Concerning the origin and pathogenesis of JNA, developmental factors, hormonal imbalance, and genetic causes are still being discussed 12…”

“…Angiofibromas with extranasopharyngeal primary localization are extremely rare lesions and different sites of origin of these tumors can be found in the international literature: maxillary and ethmoid sinus, nasal septum, middle and inferior turbinate, oral cavity, tonsil, larynx, hypopharynx, lacrimal sac, middle cranial fossa, etc 12. About 170 cases of extranasopharyngeal angiofibroma (ENA) are described in the literature and this tumor is histologically very similar, but clinically and epidemiologically very different from, to JNA 1. According to the literature, the oldest patient with ENA is 87 years, and the youngest one is 8 years.…”