An Extranasopharyngeal Angiofibroma of Mandibular Ramus in a Preschool Child - An Extremely Rare Case Report

Angiofibroma or juvenile nasopharyngeal angiofibroma (JNF) is a rare vascular benign tumour predominant in male adolescents and pre-adolescents. In spite of several origin sites reported, nasopharynx in the region of the sphenopalatine foramen and pterygopalatine fossa remains most common.1 Nevertheless, sporadic description of extra nasopharyngeal angiofibromas (ENAF) are also rarely evident.2 Literature documents maxillary sinus as the most favourable site for ENAF followed by the ethmoid sinus, nasal cavity, nasal septum, larynx, sphenoid sinus, cheek, conjunctiva, oropharynx, retromolar area and others.3 However, ENAF of mandibular ramus marks rarity and no case has been reported with respect to this anatomic location especially in preschool children yet. Hence, a rare ENAF in a 3-year-old child, principally confined to mandible, with neither sphenopalatine foramen nor nasopharynx involvement presenting with swelling is described.

Nasal Septal Angiofibroma

Juvenile nasopharyngeal angiofibroma is a benign vascular tumor seen predominantly in adolescent males in the second decade of life. Extranasopharyngeal angiofibroma includes vascular fibrous masses that occur outside the nasopharynx. The diagnosis of an angiofibroma is based on the clinical presentation and imaging, with biopsies being avoided to avoid excessive bleeding. Computed tomography scan is considered sufficient for the diagnosis of extranasopharyngeal angiofibroma as it clearly delineates and identifies the tumor.

Nasal septal angiofibroma: less vascular behaviour than observed in radiological imaging, a rare case in a 51-year-old woman

Angiofibroma of the head and neck is classically found in the nasopharynx of an adolescent male. We present a rare case where an angiofibroma was diagnosed in the anterior nasal septum of a 51-year-old woman, the eighth nasal septal angiofibroma reported in a female. This case highlights how an angiofibroma in an older woman has a less vascular behaviour than one in an adolescent male, even though radiological enhancement may suggest that the lesion is highly vascularised. Nasal septal angiofibromas can be managed by biopsy and removed by simple endoscopic resection with minimal haemorrhage. Although very rare, extranasopharyngeal angiofibroma/nasal septal angiofibroma should be included in the differential diagnosis when patients of all ages present with nasal obstruction secondary to a nasal mass, with or without epistaxis.

Diode Laser Management of Primary Extranasopharyngeal Angiofibroma Presenting as Maxillary Epulis: Report of a Case and Literature Review

Juvenile nasopharyngeal angiofibroma is a rare vascular neoplasm, mostly occurring in adolescent males, and representing 0.05% of all head and neck tumors. Nevertheless, it is usually recognized as the most common benign mesenchymal neoplasm of the nasopharynx. Usually, it originates from the posterolateral wall of the nasopharynx and, although histologically benign, classically shows a locally aggressive behavior with bone destruction as well as spreading through natural foramina and/or fissures to the nasopharynx, nasal and paranasal cavities, spheno-palatine foramen, infratemporal fossa and, very rarely, to the cranial cavity. Extranasopharyngeal angiofibroma is considered a distinct entity due to older age at presentation, different localizations (outside the nasopharyngeal pterygopalatine fossa) and attenuated clinical course. Extranasopharyngeal angiofibroma has been sporadically described in the oral cavity. We report a case of extranasopharyngeal angiofibroma with primary and exclusive involvement of the adherent gingiva of the anterior maxilla, managed by preoperative diode laser trans-mucosal photocoagulation and subsequent surgical removal. The current literature on primary extranasopharyngeal angiofibroma is also reviewed.

Frontoethmoidal Extranasopharyngeal Angiofibroma With Orbital Pyocele

Significance Statement Extranasopharyngeal angiofibromas (ENA) are rare vascular tumors that do not conform to the clinical characteristics of typical nasopharyngeal angiofibromas. We present the management of an angiofibroma in a rare site, within the frontal sinus with a concomitant orbital pyocele, which was completely excised via an endoscopic approach. ENAs should be considered as a differential diagnosis in patients with sinonasal mass and epistaxis. Awareness of this rare entity will avoid radical surgery thus decreasing postoperative morbidity.