Olfactory Neuroblastoma: Past, Present, and Future?

Abstract

OBJECTIVE: To consider the long-term survival and outcomes in patients with olfactory neuroblastoma undergoing craniofacial resection. STUDY DESIGN: A single-center prospective cohort study. METHODS: All patients with olfactory neuroblastoma treated in a 23-year period with craniofacial resection (with or without radiotherapy) were analyzed; a multivariate analysis was included. RESULTS: Forty-two patients aged 12 to 70 years were assessed, 83% of whom had received no preceding treatment. Craniofacial resection was used in all cases, combined with radiotherapy in 24 patients (57%). Duration of follow-up ranged from 2 to 206 months (mean follow-up period, 57 mo). The disease-free actuarial survival and overall survival were 77% and 61% at 5 years and 53% and 42% at 10 years, respectively. A Cox regression analysis identified intracranial extension and orbital involvement as independent factors affecting outcome. CONCLUSION: Craniofacial resection combined with radiotherapy offers the gold standard of care against which other approaches such as endoscopic resection must be judged.