Juvenile nasopharyngeal angiofibroma with intracranial extension - diagnosis and treatment

Introduction: This retrospective study analyzes radiological findings, therapeutic management and outcomes of patients with intracranial extension of JNA. The routes of intracranial spread, incidence of intracranial disease and influence on therapeutic approach are discussed. Material and methods: An evaluation on the records of 62 patients with JNA was performed and 10 patients with intracranial tumors were included in the study. All patients were males aged 10 to 19 years. Results: According to Andrews' classification 8 patients presented with stage IIIb, 1 patient stage IVa and another patient stage IVb tumor. Intracranial invasion was extradural in 8 cases and intradural in 2 patient. Surgery was performed in 9 cases and the most common was combined approach: infratemporal fossa and sublabial transantral. One patient was referred for radiotherapy. Follow-up ranged from 8 to 26 years. There was extracranial recurrence in 2 (22%) of 9 operated patients. Conclusions: The superior orbital fissure is the most frequent route of intracranial spread in patients with extensive involvement of the infratemporal fossa. Due to high risk of recurrence and potential serious complications advanced cases of JNA should be managed by experienced multidisciplinary team, preferably in tertiary referral centers, with an access to modern diagnostic and therapeutic modalities.