Esthesioneuroblastoma: Diagnosis and Treatment

Abstract

Esthesioneuroblastoma is a nasal tumor which arises from cells of neural crest origin. It is a difficult tumor to diagnose clinically and histopathologically. First described in 1924, approximately 160 cases have been reported with over 125 of these in the last 15 years. This reflects an increased awareness of the tumor by physicians rather than an increased incidence. In the past 17 years, 12 cases of esthesioneuroblastoma have been treated at the Department of Otolaryngology and Maxillofacial Surgery of the University of Virginia Medical Center. Reviewing these cases and the literature leads us to make the following recommendations for diagnosis and treatment: The diagnosis of esthesioneuroblastoma can be made by 1) the clinician who suspects it in any patient with a nasal mass causing unilateral obstruction; 2) the finding of plexiform intercellular fibrils by light microscopy (rosettes and pseudorosettes are not as common as reported); 3) the finding of secretory granules and neurites by electron microscopy of the highly undifferentiated tumors; and 4) formaldehyde-fume-induced fluorescence. Combined therapy with preoperative irradiation followed by craniofacial resection of the tumor to include the cribriform plate is recommended. This treatment should result in a five-year survival in excess of 50% of patients.