Esthesioneuroblastoma

Abstract

OBJECTIVES: To investigate the characteristics associated with survival in esthesioneuroblastoma and to determine whether the modified Kadish staging system can predict outcome. DESIGN: Retrospective population-based cohort study. SUBJECTS: All patients in the Surveillance, Epidemiology, and End Results tumor registry diagnosed as having esthesioneuroblastoma (1973-2002). MAIN OUTCOME MEASURES: The modified Kadish stage and the overall and disease-specific survival rates were determined. RESULTS: The cohort included 311 patients with a mean age of 53 years and a unimodal age distribution. The overall 5- and 10-year survival rates were 62.1% and 45.6%, respectively. The modified Kadish staging system was applied to 261 patients. Kaplan-Meier analysis showed the overall and disease-specific survival rates at 10 years to be 83.4% and 90%, respectively, for patients with stage A disease; 49% and 68.3% for patients with stage B disease; 38.6% and 66.7% for patients with stage C disease; and 13.3% and 35.6% for patients with stage D disease. Log-rank test comparisons found Kadish stage (P<.01), treatment modality (P<.002), lymph node status (P<.01), and age at diagnosis (P<.001) to be significant predictors of survival. Cox regression analysis confirmed that Kadish stage remained a significant predictor of disease-specific survival. CONCLUSION: The modified Kadish staging system, lymph node status, treatment modality, and age are useful predictors of survival in patients who present with esthesioneuroblastoma.