Juvenile Nasopharyngeal Angiofibroma: A Retrospective Institutional Surgical Case Series

Background: Juvenile nasopharyngeal angiofibromas (JNA) are highly vascular benign tumors that originate in the pterygoid space and often involve multiple compartments. It is the most common tumor presenting in the nose and nasopharynx and is seen exclusively in young men, typically between the ages of 9 and 19 years. Surgery remains the main treatment modality, particularly when coupled with preoperative embolization. We report our experience with a series of 20 patients with JNA treated between 2005 and 2019.

Methods: After Institutional Review Board approval, a retrospective review was preformed to identify all patients with JNA with surgery at our institution during the study period 2005–2019. Only those patients who underwent either primary or secondary surgical treatment at our institution and had a confirmed pathologic diagnosis of angiofibroma were included in the study. Data abstraction included demographics, clinical presentation, operative technique, and postoperative outcomes.

IRB 19–008145.

Results: Twenty patients, all males, were included, with an overall median age at diagnosis of 18 years (range, 9–26). Sixteen (80%) patients received primary diagnosis and treatment at Mayo Clinic, and four (20%) were treated after previous unsuccessful treatment at another institution The most common presenting symptoms were nasal obstruction in 15 (75%) epistaxis in 14 (70%), and headache/facial pain in 5 (20%). In 65% of the cases, the tumor was right sided. All patients underwent preoperative embolization before surgical resection. The internal maxillary artery was predominant in all 20 patients. All patients were operated within 24 to 48 hours following embolization. Tumor size (maximum dimension) ranged from 1.6 to 10.7 cm with a median size of 4.4 cm. Based on the Radkowski classification, there was two (10%) stage IA, three (15%) stage IB, two (10%) stage IIA, five (25%) stage IIIB, seven (35%), and one (5%) stage IIIB. All tumors were confirmed as JNA on pathological analysis. In 16 patients (80%), an endoscopic approach was used and 4 patients (20%) had open approaches. Gross total resection was achieved in all patients. One patient (5%) presented with recurrence. This patient had an atypical angiofibroma and required multiple interventions. There were no mortalities associated with the procedures. One patient (5%) had a postoperative CSF leak that was managed with reconstruction of the middle fossa and a lumbar drain. The average estimated blood loss was 736 mL (range: 50–6750 mL). Two patients required blood transfusions (10%). No patient underwent radiotherapy or radiosurgery.

Conclusion: The EEA is a safe and effective technique even for large JNAs. A multidisciplinary team consisting of a skull base neurosurgeon and an otorhinolaryngologist with extensive expertise in endoscopic surgery is required for optimal results.