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This study aims at evaluating the incidence and clinical features of extranasopharyngeal angiofibroma (<jats:styled-content style=\"fixed-case\">ENA<\/jats:styled-content>).<\/jats:p><\/jats:sec><jats:sec><jats:title>Material and Methods<\/jats:title><jats:p>Systematic review of the literature (Medline<jats:sup>\u00ae<\/jats:sup> and Google<jats:sup>\u2122<\/jats:sup>) up to 31 December 2015.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>174 cases of <jats:styled-content style=\"fixed-case\">ENA<\/jats:styled-content> 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\u00a0years old (mean: 28.7\u00a0years; median: 23\u00a0years). 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\u00a0months on average (median: 4\u00a0months). Brisk bleeding resulted in 11 of 43 biopsy procedures. Surgical resection as first\u2010line therapy was performed in 170 patients. A tumour regrowth within 12\u00a0months was registered in four patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The increasing awareness of <jats:styled-content style=\"fixed-case\">ENA<\/jats:styled-content> and the willingness to publish case reports\u2014not only in Medline\u2010listed journals\u2014resulted in a significant increase of published case reports lately. Although extremely rare, <jats:styled-content style=\"fixed-case\">ENA<\/jats:styled-content>s 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 <jats:styled-content style=\"fixed-case\">ENA<\/jats:styled-content> in their differential diagnosis of any mass of the upper airway.<\/jats:p><\/jats:sec>","DOI":"10.1111\/coa.12939","type":"journal-article","created":{"date-parts":[[2017,7,17]],"date-time":"2017-07-17T05:26:36Z","timestamp":1500269196000},"page":"199-222","source":"Crossref","is-referenced-by-count":36,"title":["Extranasopharyngeal angiofibroma revisited"],"prefix":"10.1111","volume":"43","author":[{"given":"J.P.","family":"Windfuhr","sequence":"first","affiliation":[{"name":"Department of Otorhinolaryngology, Head and Neck Surgery Hospital of Maria Hilf Moenchengladbach Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5071-9580","authenticated-orcid":false,"given":"J.","family":"Vent","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology Medical Faculty University of Cologne 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