Resection of Juvenile Nasopharyngeal Angiofibroma (JNA): A Mayo Clinic Experience

Introduction: Juvenile nasopharyngeal angiofibroma (JNA) is a rare vascular tumor that is almost exclusively found in adolescent males. Endoscopic endonasal approaches reduce morbidity compared to open approaches, but limited data exists concerning patient and tumor characteristics in selection of surgical approach. In this case series, we aimed to assess associations between tumor location and volume in selection of surgical approach and incidence of JNA recurrence.

Methods: A retrospective chart review of patients who underwent JNA resection between January 2010 and January 2024 was performed. Variables included demographics, radiographic tumor volume ( cm³), UPMC staging, surgical approach, and pathologic recurrence. UPMC Stage I denotes a tumor located within the nasal cavity, specifically the medial pterygopalatine. Stage II is a tumor that has grown into the paranasal sinus, specifically the lateral pterygopalatine fossa and has no residual vascularity. Stage III is a tumor that causes skull base erosion or located in the orbit, infratemporal fossa, and has no residual vascularity. If a stage III tumor has evidence of residual vascularity, then this is denoted stage IV. Stage V is a tumor with intracranial extension and with residual vascularity. We performed descriptive statistics and correlation studies.

Results: Of the 24 patients who met the inclusion criteria, all patients were male with a median age of 24 years (range: 14–35). Twenty-two patients (92%) presented for primary resections, while 2 (8%) were revision surgeries. The median JNA radiographic tumor volume was 51.8 cm³ (range: 8.7 to 190.0 cm³). UPMC stages I, II, III, IV, and V were 3 (13%), 8 (33%), 2 (1%), 10 (42%), 1 (1%), respectively. Endoscopic endonasal approach was the most common surgical approach with 20 patients (83%), while 4 (17%) underwent a combined open and endoscopic approach. Primary tumor volume was not significantly associated with surgical approach taken (p = 0.62). There is correlation between UPMC staging and radiographic tumor volume, suggesting that increased UPMC staging is due to greater volume and local extension (r = 0.44 [95% CI: 0.04–0.71]; p = 0.03). Additionally, patients requiring combination endoscopic and open surgery were more likely to exhibit a higher UPMC staging (2.7 vs. 4.3; p = 0.012) compared to patients treated solely by endoscopic surgery. Of the 4 patients who underwent combination open and endoscopic approaches, 0 (0%) experienced recurrence of disease while 5 (25%) patients treated endoscopically had recurrence. Lastly, primary tumor volume (p = 0.09), UPMC staging (p = 0.57), and surgical approach (p = 0.32) were not associated with increased risk of recurrence.

Conclusion: In this case series, higher UPMC stages were associated with a combination surgery approach rather than endoscopic surgery alone. Tumor volume did not impact the type of surgical resection or recurrence rate. Tumor location, particularly as determined by UPMC staging, was likely the main factor influencing the chosen surgical approach. Collectively, these data suggest that patients with higher UPMC stages may require a combination approach for difficult-to-access tumors, potentially justifying added morbidity for reduced recurrence rates.