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juvenile angiofibroma
Wednesday 8 September 2004
Definition: Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign neoplasm of the nasopharynx that accounts for 0.5% of all head and neck tumors.
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Juvenile nasopharyngeal angiofibroma (JNA)
Although histologically benign in appearance, JNAs are locally aggressive and destructive, spreading from the nasal cavity to the nasopharynx, paranasal sinuses, and orbit skull base with intracranial extension.
The gender selectivity of JNA and the relatively young age at diagnosis suggest hormone-dependent development.
Hormonal disorders have been reported in patients with JNA, and androgen and estrogen receptors have been identified in tumor tissue; however, a hormonal influence on JNA is controversial.
Immunochemistry
beta-catenin immunostaining (11238055)
Etiology
frequent somatic mutations of CTNNB1 coding for beta-catenin (11238055)
Predisposition
familial adenomatous polyposis (FAP - APC mutations) (18227724, 15951557)
Variants
giant cell angiofibroma recurring as a solitary fibrous tumor (18645449)
References
Coutinho-Camillo CM, Brentani MM, Nagai MA. Genetic alterations in juvenile nasopharyngeal angiofibromas. Head Neck. 2008 Mar;30(3):390-400. PMID: 18228521
Valanzano R, Curia MC, Aceto G, Veschi S, De Lellis L, Catalano T, La Rocca G, Battista P, Cama A, Tonelli F, Mariani-Costantini R. Genetic evidence that juvenile nasopharyngeal angiofibroma is an integral FAP tumour. Gut. 2005 Jul;54(7):1046-7. PMID: 15951557
Abraham SC, Montgomery EA, Giardiello FM, Wu TT. Frequent beta-catenin mutations in juvenile nasopharyngeal angiofibromas. Am J Pathol. 2001 Mar;158(3):1073-8. PMID: 11238055
