Esthesioneuroblastoma: a population-based analysis of survival and prognostic factors.

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.