Surgical Management of Olfactory Neuroblastoma: Outcomes and Patterns of Failure

Abstract

Objectives:

(1) Analyze survival, complications, and impact of transnasal endoscopic cranial base surgery. (2) Describe time course and patterns of local, regional, and distant failure patients with olfactory neuroblastoma (ONB) of the nasal cavity, ethmoid sinus, and/or anterior skull base.

Methods:

Review of prospectively collected clinical data of patients treated by a single head and neck surgeon at 2 academic medical centers over 16 years.

Results:

Thirty-seven patients were diagnosed with ONB confirmed by immunohistochemistry between 1996 to 2010, and 31 are available for review. There were 10 women and 21 men with a mean age of 50.9 years. Patients were treated with primary resection and no additional therapy (4), radiation therapy (24), or chemotherapy enhanced radiation therapy (3). Primary resections included open medial maxillectomy without craniotomy (n = 2), endoscopic anterior craniofacial resection (CFR) (n = 9), and open CFR (n = 20). Mean and median overall survivals were 95.95 and 80.62 months, respectively (range, 6.53 to 242 months). Operative complications were only observed in patients undergoing open CFR. Recurrences were either limited to the neck (3/11), the neck and a distant site (3/11), or distant sites alone (5/11). Time interval to the development of recurrence ranged from 3 to 186 months, and only patients with isolated recurrences could be salvaged. Intradural tumor extension correlated with recurrence but not necessarily early death.

Conclusions:

Margin negative resection of ONB should remain the goal of primary treatment. Long-term follow-up is necessary to identify patients with isolated recurrences who would benefit from aggressive salvage therapy.