Olfactory Neuroblastoma: Long-Term Outcomes

Abstract

Objective: Olfactory neuroblastomas (ON) are rare malignant tumors of neuroectodermal origin arising from neuroepithelium in the upper part of the nose. Our objective was to present long-term outcomes in patients with ON. Methods: A retrospective analysis of all cases referred to a tertiary referral center over the past 15 years was performed. Demographics, staging at presentation, management and outcomes were examined. Results: Nine patients were identified. The mean age was 54 years (range 43-64 years) with a male to female ratio 5:4. At presentation there was one stage A, two stage B, and six stage C ONs. One case was treated with endoscopic resection and radiotherapy, three cases with endoscopic resection alone, one case with craniofacial resection, two cases with craniofacial resection and radiotherapy, and the remaining two cases were treated with craniofacial resection and chemoradiotherapy. Recurrence rates reached 56% but these were late in most cases, occurring 5.2 years after the primary treatment (range 3-13 years). Recurrence was treated with further surgery, radiation or combination. Average survival was 6.4 years (range 2-14 years). No patient has died from the disease itself in a mean follow-up of 7 years (range 2-14 years). Conclusion: All treatment modalities were well tolerated in patients with ON with high patient survival rates. Recurrence is not uncommon in a later stage but it can still be managed with individualized approach.