Combined modality therapy of esthesioneuroblastoma.

Abstract

Esthesioneuroblastoma (ENB) is a rare tumor of the olfactory epithelium. The objective of this study was to evaluate treatment modalities including surgery, IMRT, and chemotherapy and patient outcomes. A retrospective analysis was performed on a total of 21 patients. Therapy included craniofacial resection (CFR), radiotherapy, chemotherapy, or a combination of these methods. The median follow-up period was 47 months. Surgery was performed in 90.4% of cases; radiotherapy was performed adjuvantly in 15 (72.7%) patients. Surgery, radiotherapy, and chemotherapy were administered to 7 (33.3%) patients. Eight (38.3%) patients had local recurrence. The 5-year crude overall survival was 71.4% and actuarial 5-year overall survival was 58% with confidence interval (CI, 25 and 81, respectively). The 5-year crude disease-free survival rate was 59% and the 5-year actuarial disease-free survival rate was 62% (CI, 28 and 83, respectively). Multidisciplinary therapy of ENB should be considered, especially for Kadish C and high-grade lesions. Craniofacial resection (CFR), Intensity modulated radiation therapy (IMRT), and chemotherapy should be investigated in a multi-institution trial of ENB.