Esthesioneuroblastoma

Abstract

Esthesioneuroblastomas (ENB) are rare malignant tumours of neuroepithelial origin arising from the olfactory mucosa. Not uncommonly they metastasize to regional lymph nodes and distant organs. Commonly they have a long and insidious course and the diagnosis requires a high index of suspicion. Since this tumour was first described, many grading and staging systems have been proposed but still there is some controversy on which correlates best with tumour prognosis. The classic treatment for ENB is craniofacial resection with the goal of gross total resection and achievement of tumour-free margins. The enormous advances in endoscopic techniques over the last two decades means this now has comparable results in achieving tumour-free margins, especially for low stage tumours. The role of preoperative and postoperative radiation has been well established in the treatment of this tumour. Controversy exists regarding the role of neck dissection or elective neck radiation as standard therapeutic modalities.