Esthesioneuroblastoma

Abstract

Esthesioneuroblastoma is an uncommon tumor of neural crest origin arising in the nasal cavity. Since 1966, 97 cases have been reported in the world literature. In this report an analysis is presented of the following aspects of these 97 patients: age and sex distribution, disease staging, treatment results, interval to recurrence, and survival. Staging was according to a system proposed by Kadish et al. There is a bimodal age distribution with peaks in age groups 11-20 years and 51-60 years. Prognosis is favorable in early stage disease (A and B) with 3-year crude survivals of 88.9% and 83.3%, respectively. Stage C patients have a poor prognosis, with 52.9% surviving 3 years. Treatment consisted of radiotherapy, surgery, or a combination of radiotherapy and surgery. In stage A, prognosis is favorable in all three treatment groups with one 1 of 24 patients dying of recurrent disease. In stage B, recurrence rates and 3-year survivals are also similar in the three treatment groups. Thus, single modality treatment is as effective as combined treatment for early stage disease. Local recurrence is the predominant site of failure occurring in 68% of recurrences. The overall crude survival for the 97 patients was 95.4%, while the determinate survival (corrected for intercurrent disease) was 70.8%.