Juvenile primary extranasopharyngeal angiofibroma, presenting as cheek swelling

Abstract

Angiofibroma is a locally advancing immensely vascular tumor that essentially arises from the nasopharynx. The clinical characteristics of extranasopharyngeal angiofibroma (ENA) do not accord to that of nasopharyngeal angiofibroma and can present a diagnostic confront. We describe a case of primary juvenile ENA in a 19-year-old patient who presented with a rapidly enlarging mass of the cheek region. The case is unusual because of its anatomic location. The diagnostic and management particulars are sketched.

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“…3 APC and beta-catenin alterations, along with the in uence of testosterone, may accompany this lesion's development. 10 Aneuploidy is notably absent in ENA, as chromosomal gains are proposed to activate oncogenes without concomitant loss of tumor suppressor genes. Inconspicuous endothelium, fragile thin walled blood vessels, and a prominent brous stroma collectively contribute to the diagnosis and distinguish ENA from similarly appearing lesions.…”

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

“…Conversely, JNAs with maxillary involvement, with or without extension into the oral cavity, are more easily diagnosable, and an incisional biopsy is performable in most cases. Nevertheless, the differential diagnosis remains extremely challenging, especially for the presence of undefined radiological borders, thus including benign/malignant epithelial and odontogenic neoplasms, benign and malignant maxillary/nasal sinuses neoplasms, localizations of hematological malignancies and metastatic tumors [ 6 , 36 , 37 , 38 , 39 , 40 , 41 ].…”

“…ENA also displays a poorer blood vessel component and more intense fibrosis at histological examination, and less propensity for recurrence after surgical treatment. [ 2 , 3 , 13 , 23 , 36 , 42 , 43 ].…”

“…This may frequently be the case for angiofibromas involving the cheek and manifesting as facial swellings but taking their origin in the nasal/maxillary cavities or in the pharynx. ENAs involving the maxilla, with or without gingival manifestations, usually are extensions of a tumor arising in the nasal or maxillary cavities [ 36 , 37 , 42 ] In this regard, in 2005 our group published the case of an angiofibroma with primary intra-oral presentation [ 37 ] localized in the posterior maxillary gingiva of a young girl, but with involvement (and most probable origin) in the maxillary sinus. Therefore, we concur with Windfuhr et al, who stated that “It would be ideal to differentiate histologically and demographically between intranasal and other locations of extranasopharyngeal angiofibromas (ENA) when analysing these tumours in the future.…”

“…The case reported herein show overlapping clinical features with previously reported ENAs, as occurring in an adolescent male (17 years old), showing bleeding at the first attempt of surgical removal and representing a diagnostic challenge based on its clinic-radiological presentation. In fact, the peripheral localization, along with the radiological appearance, pointed at a wide range of clinical diagnoses including several reactive or neoplastic lesions with gingival/periodontal onset (such as periodontal infections, proliferative osteitis, exostosis, peripheral osteoma, peripheral ossifying fibroma, giant cell granuloma, and lobular capillary hemangioma) [ 13 , 36 , 37 , 38 , 42 , 44 , 45 , 46 ].…”