Juvenile nasopharyngeal angiofibroma from an epidemiological perspective

Abstract

Introduction: Juvenile nasopharyngeal angiofibroma is a benign, vascular, and locally aggressive tumor that almost exclusively affects adolescent males. It accounts for between 0.05% and 0.5% of head and neck tumors, and although histologically benign, its clinical presentation can be infiltrating and cause severe complications.

Objective: To conduct a narrative review of the epidemiology, pathophysiology, imaging, classification, surgical approach, preoperative embolization, and recurrence of juvenile nasopharyngeal angiofibroma. Methods:  A search was performed in PubMed, Scopus and Google Scholar to identify narrative reviews, systematic reviews, case series and original articles in English and Spanish published between 1996 and 2024. References and recent reviews on surgical management, embolization and molecular basis of juvenile nasopharyngeal angiofibroma were included.

Analysis of information: This disease has an estimated incidence of 1 in 150,000 inhabitants. Its etiology is related to somatic mutations in CTNNB1 (β-catenin), as well as alterations in the APC gene in cases associated with familial adenomatous polyposis. Diagnosis is based on typical clinical features, nasoendoscopy, and imaging. Standard treatment is endoscopic surgical resection, preceded by preoperative embolization.

Conclusions: Juvenile nasopharyngeal angiofibroma is a rare but clinically relevant entity. The integration of early diagnosis, selective embolization, and endoscopic approach has significantly improved surgical outcomes and reduced recurrence rates in early stages.