Advanced craniofacial juvenile nasopharyngeal angiofibroma. Description of surgical series, case report, and review of literature

Abstract

Background: Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign tumor occurring almost exclusively in adolescent and young adult males. The tumor is characterized by slow progression, aggressive growth, high vascularization, and increased rate of persistence and recurrence. The aim of this study was to describe a case of giant JNA from our practice and discuss the controversies of surgical treatment of advanced JNA.

Material and methods: A series of 29 consecutive male patients with JNA Fisch grade III and IV was surgically treated in Burdenko Neurosurgical Institute from 2000 until 2008. In the vast majority of cases, endovascular embolization and surgical removal via orbitozygomatic approach were applied.

Results: Gross total resection was achieved in 24 cases (83%). Complications were encountered in eight cases. No mortality was observed. In three patients, the diseases recurred. An illustrative case is described.

Conclusion: Surgical treatment is the basic tactics in management of extensive JNA including endovascular embolization and resection of the tumor. We recommend using orbitozygomatic approach or its modifications in JNA. Radiation therapy may be recommended for patients with small residual tumor.