Esthesioneuroblastoma

Esthesioneuroblastoma, also termed olfactory neuroblastoma, is a rare malignancy of neural crest origin, which comprises about 6% of nasal cavity and paranasal sinuses tumors. First described by Berger and Luc in 1924, more than 1400 cases have since been reported in the literature. The neoplasm develops from the olfactory epithelium of the cribriform plate, the superior upper one-third of the nasal septum, and the upper surface of the superior turbinate, and has a propensity to invade the skull base and intracranial space. Esthesioneuroblastoma has been known by many names including esthesioneurocytoma, olfactory intranasal neuroblastoma, esthesioneuroepithelioma, olfactory esthesioneuroma, tumor of olfactory placode, and neuroolfactory tumor. The rarity of this tumor has led to the slow progress in our understanding of its pathobiology and the lack of consensus regarding treatment.