Intracranial extension of juvenile nasopharyngeal angiofibroma: patterns of involvement with a proposed algorithm for their management.

Abstract

Objective: The objective of this study was to discuss the characteristics of intracranial extension in patients with juvenile nasopharyngeal angiofibroma (JNA) and propose and an algorithm for its management.

Methods: A retrospective chart review of all patients with JNA who underwent operations between January 2013 and January 2023 was done, and those cases with intracranial extension categorized as stage IIIb, IVa, and IVb according to the Andrews modification of the Fisch staging classification were included in the study. Data were collected about age at presentation, symptoms, radiological findings, routes of intracranial extension, therapeutic management, and follow-up.

Results: Of 142 patients who underwent surgery for JNA, there were 40 (28.2%) cases with intracranial involvement. All patients were male with ages ranging from 10 to 26 years, with a mean age of 17 years at presentation. According to Andrews-Fisch classification, 28 patients presented with stage IIIb, 10 patients with stage IVa, and 2 patients with stage IVb. Parasellar involvement via the superior orbital fissure was the most frequent route of intracranial spread in patients with extensive involvement of the infratemporal fossa. All patients underwent surgery, and the most common approach was endoscope-assisted midface degloving. A total of 4 patients underwent craniotomy with an endoscope-assisted transfacial approach, which was single-stage surgery in 2 patients and a staged procedure in 2 patients. Blood transfusion was required in 53.6% of stage IIIb, 90% of stage IVa, and 100% of stage IVb patients. Residual tumor was present in 4 patients, and 3 patients developed recurrent disease. Postoperative radiotherapy was given to 5 patients. An algorithm for the surgical management of JNA with intracranial involvement was proposed on the basis of the authors' results.

Conclusions: In most cases, JNA with extradural intracranial extension can be completely excised with an endoscopic or endoscope-assisted transfacial approach, but a tumor with intracranial intradural extension requires tailored craniotomy along with a transfacial approach that can be done in single sitting or as a staged surgery. A small number of patients with gross cavernous extension receiving blood supply from a cavernous segment of the internal carotid artery are better suited for Gamma Knife or intensity-modulated radiation therapy of the residual lesion in the cavernous sinus.