Olfactory neuroblastoma: past, present, and future?

Abstract

To consider the long-term survival and outcomes in patients with olfactory neuroblastoma undergoing craniofacial resection. A single-center prospective cohort study. 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. 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. Craniofacial resection combined with radiotherapy offers the gold standard of care against which other approaches such as endoscopic resection must be judged.