Extranasopharyngeal angiofibroma of the sinonasal tract: A systematic review

PURPOSE: Extranasopharyngeal angiofibromas of the sinonasal tract (ENA-SNT) are exceptionally rare tumors that remain poorly characterized in the literature. We aim to systematically review and synthesize the clinical characteristics, management, and outcomes of ENA-SNT. METHODS: This review adhered to PRISMA guidelines. A literature search was conducted across major databases, including PubMed, EMBASE, Web of Science, and Google Scholar. Articles that reported ENA-SNT cases in humans were eligible for inclusion. Data extraction and quality assessment were independently performed by two reviewers. RESULTS: A total of 145 studies were included, comprising 163 patients. The mean age was 28.8 years (range, 0-78), with a male-to-female ratio of 2.1:1. The nasal septum (33.3%), inferior turbinate (16.6%), and maxillary sinus (16.0%) were the most frequent tumor sites. Common symptoms included nasal obstruction (73.0%) and epistaxis (68.1%), often in combination (55.2%). Angiography demonstrated no hypervascularity in 23.1% of cases; when present, the internal maxillary artery supplied the tumor in 96.1%. Preoperative biopsy caused brisk bleeding in 44.5%. Surgery was the primary treatment in 94.4% of cases, with a recurrence rate of 5.3% and an average time to recurrence of 3.8 months. Intracranial and orbital involvement were rare: 1.84% and 2.45%, respectively. CONCLUSION: This review supports ENA-SNT as a clinical entity distinct from juvenile nasopharyngeal angiofibroma. Symptoms develop more rapidly, but the tumor is less aggressive, less vascularized, and has a better prognosis. ENA-SNT can occur across all age groups and in female individuals. Surgical resection is the treatment of choice, with low recurrence rates.