Esthesioneuroblastoma.

Esthesioneuroblastoma is a rare tumor for which the published literature does not provide an evidence-based consensus treatment approach. Multimodality therapy including a combination of surgery and radiation appears to provide the best disease-free and overall survival. Surgical resection should proceed through a craniofacial approach if possible. The role of chemotherapy in the initial treatment paradigm is less clear. Chemotherapy should not be used as single-modality therapy for initial treatment but may provide additional benefit when used in combination with radiation and surgery, particularly in advanced-stage disease. Combination chemotherapy should be considered as initial therapy for unresectable tumors and metastatic disease, and as salvage therapy in disease recurrence.