Juvenile Nasopharyngeal Angiofibroma: Symptoms, Staging, and Management
Histologically benign but locally aggressive, angiofibroma is a vascular tumor of nasopharynx that exclusively affects adolescent boys. It is locally invasive and hence, can invade adjacent anatomical structures such as nose, cheek, orbit, or brain, giving rise to characteristic ‘frog-face’ deformity. The patient typically presents with progressive nasal obstruction and profuse epistaxis. Due to expansion of tumor, secondary signs and symptoms arise such as neck swelling, hyposmia and anosmia, diplopia, facial pain, and headache, among others. After clinical examination, confirmed diagnosis is based on CT findings which exhibit characteristic ‘Hollman-Miller Sign’. Digital Subtraction Angiography (DSA) is carried out to determine the feeding artery to the tumor. Andrew’s Staging System for Angiofibroma classifies tumors as: Type 1: Limited to nasopharyngeal cavity Type 2: Invasion of pterygopalatine fossa and/or maxillary, sphenoid, or ethmoid sinuses Type 3a: Invasion of infratemporal fossa without intracranial involvement Type 3b: Invasion of infratemporal fossa with intracranial extradural involvement Type 4: Invasion of intracranial, intradural tumors +/- infiltration of cavernous sinuses, optic chiasm, or pituitary fossa Andrew’s stage 1, 2, and 3a are managed by endoscopic resection of tumor. Stage 3b and 4 are difficult to manage due to invasion of structures; tumor may be approached sphenoid sinus or through orbital fissures. Pre-operative embolization is imperative in all cases except very small tumors, in order to minimize blood loss. Source - Juvenile Angiofibroma: Current Management Strategies https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5796815/ - https://www.researchgate.net/figure/Andrews-et-al-10-staging-system-for-juvenile-angiofibroma_tbl2_42588229 Image via - http://otolaryngology.wdfiles.com/local--files/rhinology/66022256-jna.pdf - https://www.sciencedirect.com/science/article/pii/S2090074016300810