ENPA has been stratified to nodules consisting of blood vessels and fibrous tissue, happening outside the nasopharynx [3]. Since the spread, gender, age, site of lesion, pathogenesis, clinical and imaging features, and recurrence are totally different from NAs [1, 4], ENPA must be observed as a distinct clinical existence [3]. Older patients are involved, females can also be affected, symptoms develop more speedily, and hypervascularity is minimal [3]. ENPA presents in an older cohort more than NA, with an average age of 22 years and 17 years, respectively, whereas for nasal vestibule angiofibroma the average age is 43 years [6]. While NA dominantly affects males, ENPA is a major happening in females [6]. The clinical manifestation of ENPA relies on the site and extent [3]. NA rises from the region of the pterygoid plate and the sphenopalatine foramen, and its etiology is debatable. Migration error of the basal fascia is considered as the etiology of ENPA, justifying its existence in diverse sites [4]. The most typical location is the maxillary sinus [4] while the septum is a very rare location [1].

Spinosi et al. reported in 2017 a case of a nasal septum angiofibroma in a 28-year-old man who suffered from right-sided epistaxis, airflow impairment, and nasal swelling [1]. Tasca and Compadretti reported in 2008 a case of a 57-year-old woman with a 1-year history of a right nasal obstruction due to an angiofibroma adhering to the posterior nasal septum [3]. Baptista et al. reported in 2014 a case of an 8-year-old girl who suffered from bilateral nasal obstruction and recurrent epistaxis, worse on the right side, with hyposmia and snoring due to ENPA inserted in the right inferior turbinate [4]. A rare case of angiofibroma of the mandible in a 16-year-old female patient was reported in 2016 by Khaliq et al. [7].

Though the management of ENPA is not totally codified and even the histopathological examination emphasizes the diagnosis, ENPA can be efficiently diagnosed and managed early with imaging, endoscopy, and clinical features, while embolization has been used in selected cases [1]. CT and MRI are essential to define the neoplasm’s location and extension, with focusing on skull base involvement, intracranial extent, and relation to significant vessels and neurologic entities [3, 6]. Using a contrast agent with CT and MRI in NA produces a strong and commonly homogeneous enhancement. ENPAs generally have moderate enhancement of contrast or even nothing, due to the usual poor vascularity [3, 6]. Signs of suspected hypervascularity; indicate the need for arteriography before surgery to organize the needful precautions and minimize the risk of severe bleeding throughout biopsy or neoplasm eradication [3]. Using angiography and eclectic embolization must have a high threshold, that is, only if there is radiological proof of widespread, massive and hypervascular lesion [6].

Surgery is the treatment of choice [1, 3] with often preoperative embolization [7]. Although the maxillary artery supplies ENPA such as NA, it might not cause exaggerated bleeding during surgery due to the predominance of fibrous stroma, unlike NA [4]. An endoscopic and endonasal eradication, under general anesthesia, must be attempted, though too much bleeding could demand an external incision. Electrical cauterization may not suffice, so an abrasion from the submucosal to the sub-perichondral area must be performed [1]. Other treatment modalities include [7]: sclerosing agents, radiotherapy, preoperative hormone therapy, chemotherapy, and arterial ligation. The outlook therapy should contain intraarterial immunotherapy and specific drugs that inhibit angiogenesis [7].

Differential diagnosis; includes hemangioma, hemangiopericitoma, or pyogenic granuloma [4]. The recurrence ratio of NA ranges from 6 to 27.5%, whereas no recurrence was notified for ENPA [4, 6] because its extrapharyngeal site simplifies total resection [4].

Our patient had an age of onset and the site of lesion different from those of most ENPAs. As usually occurs, epistaxis was the presenting sign. Under general anesthesia and endoscopically, the mass was removed en bloc without any significant intraoperative blood loss and without requiring preoperative embolization. Follow-up appointments confirmed no recurrence over 1 year after surgery.

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