Esthesioneuroblastoma

Thirty-nine patients with esthesioneuroblastoma are reviewed. The presentation of the tumor, symptomatology, investigation, and treatment are discussed. A recommended treatment regimen is outlined. Histologic typing is valueless in predicting tumor behavior. An illustrative and difficult case of recurrent base of skull esthesioneuroblastoma is presented. The resection performed is described, and the problem of extradural oropharyngeal communication is discussed. The solution was to use a temporalis and galeal frontalis flap. Reconstruction was with an external and intraoral prosthesis. Optimal treatment in a fresh lesion is radical surgery with or without radiation therapy. Esthesioneuroblastoma is a rare and often misdiagnosed malignant tumor of the olfactory epithelium. Originally described by Bergen et al. in 1924 as "esthesioneuroepithelioma olfactif," it was introduced into the North American literature by Schall and Lineback in 1951. Since then, fewer than 200 cases have been collected. The various terms used to describe it--olfactory esthesioneuroblastoma, esthesioneurocytoma, and olfactory neuroblastoma--all denote origin from the neural crest. The sensory nerves of smell are short bundles of fibers that originate in the olfactory bulb and pass through the cribriform plate to the olfactory area of the nasal mucosa. This mucosa is located in the most superior part of both nasal fossae. Thus the usual primary sites of occurrence include the superior nasal cavity or nasal septum, and turbinates, the ethmoid, or the cribriform plate, although an extranasal site of origin has been suggested. Symptoms are usually progressive and range from nasal obstruction or epistaxis to diplopia, ocular pain, and headaches in the more advanced disease state.(ABSTRACT TRUNCATED AT 250 WORDS)