An Aggressive Juvenile Nasopharyngeal Angiofibroma With Intracranial and Orbital Extension in an Adolescent Female: A Case Report and Focused Literature Review

Juvenile nasopharyngeal angiofibroma (JNA) is a rare, benign but locally invasive vascular tumor of childhood that almost exclusively affects adolescent males. We present a very unusual case of a 14-year-old female with JNA, who presented with persistent headache, nasal obstruction, post-nasal drip, and intermittent epistaxis. Contrast-enhanced CT imaging revealed a highly enhancing mass located in the sphenopalatine fossa, coursing into the left nasal cavity, nearby soft tissues, paranasal sinuses, and eroding the hard palate. Additional imaging showed that the mass extended into the orbit and intracranium, occupying the left middle cranial fossa. The patient underwent multidisciplinary surgical management by otorhinolaryngologists and neurosurgeons. This case contributes to the sparse literature on JNA in genetically female patients. Although rare female cases have been reported, most angiofibromas in females have occurred post-menopause or have arisen from atypical extra-nasopharyngeal locations. This instance is unique in that we demonstrate a classic nasopharyngeal origin in an adolescent female, which adds to the current literature on the atypical population demographics of JNA. A review of past female cases further supports the conclusion that physicians should include JNA in the differential diagnosis for vascular nasopharyngeal masses, regardless of sex.