Esthesioneuroblastoma: diagnosis and management.

Esthesioneuroblastoma is an uncommon nasal tumor originating from cells of neural crest origin. An increased awareness of this neoplasm by clinicians and pathologists has led to an increase in its diagnosis. In the Department of Otolaryngology--Head and Neck Surgery at the University of Virginia Medical Center, 22 cases have been treated. A review of the biologic course of these cases has allowed us to make certain recommendations for therapy. Patients with stage A and B disease are best treated with preoperative radiation therapy followed by craniofacial resection. Patients with stage C disease are treated with cyclophosphamide and vincristine before irradiation and craniofacial resection. If a significant response occurs these patients receive postoperative chemotherapy. We believe this treatment should result in five-year survivals in excess of 50%.