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Extranasopharyngeal Angiofibroma

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Juvenile Angiofibroma

Abstract

Angiofibromas in the head and neck area are acknowledged to originate from the superior aspect of the sphenopalatine foramen, typically presenting in male adolescents, and to grow with a highly predictable spreading pattern. They may also originate from or localized elsewhere than the nasopharynx, commonly labeled extranasopharyngeal angiofibroma (ENA). A review of the current literature indicates that the nasal septum is most commonly involved in the disease besides the maxillary sinus, inferior tubinate, ethmoid sinus, nasal cavity, and various other sites. ENA presents late ata median age of 23 years with a more balanced sex ratio compared to juvenile angiofibromas. Symptoms develop shortly in a median time of 4 months, most commonly as nasal obstruction, either in combination with epistaxis (25.8 %) or other symptoms (12.6 %). Surgery is rarely complicated by bleeding requiring major surgical approaches or preoperative embolization. Transnasal or transmaxillary resection is feasible in most cases and multidisciplinary approaches rarely indicated. Tumor recurrence is extremely rare and usually occurs within the first postoperative year. Although extremely rare, ENAs have to be encountered in the differential diagnosis, particularly if adult patients present with a rapidly developing nasal obstruction resulting from a nasal septum tumor. Female gender or normal vascularity does not exclude diagnosis. Further research is indicated to clarify the origin of this rare entity.

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  1. Department of Otorhinolaryngology, Plastic Head and Neck Surgery, Kliniken Maria Hilf, Sandradstraße43, 41061, Mönchengladbach, Germany

    Jochen P. Windfuhr MD

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  1. Department of Otolaryngology-Head and Neck Surgery, Port Moresby General Hospital and School of Medicine and Health Sciences, University of Papua New Guinea, Boroko Port Moresby, Papua New Guinea

    Siba P. Dubey

  2. Department of Otolaryngology-Head and Neck Surgery, University of Saarlands Medical Center, Homburg/Saar, Saarland, Germany

    Bernhard Schick

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Windfuhr, J.P. (2017). Extranasopharyngeal Angiofibroma. In: Dubey, S., Schick, B. (eds) Juvenile Angiofibroma. Springer, Cham. https://doi.org/10.1007/978-3-319-45343-9_21

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