Purpose of review: This article provides a contemporary review of the management of esthesioneuroblastoma (ENB) within the context of recent surgical advancements, while highlighting the ongoing limitations and controversies surrounding this uncommon pathologic entity.
Recent findings: Surgical management of ENB is continuously evolving with contemporary endoscopic approaches complementing or in many cases replacing open approaches.
Summary: Complete surgical resection remains the mainstay definitive therapy for ENB. Aggressive, combined modality therapy appears to be justified in patients at greatest risk of developing recurrence based on advanced tumor stage and high pathologic grade. ENB requires prolonged surveillance following treatment given its tendency for late recurrence.