Pediatric sinonasal tumors in the United States: incidence and outcomes.

BACKGROUND: Sinonasal tumors in the pediatric population are exceedingly rare.

MATERIALS AND METHODS: Surveillance, Epidemiology, and End Results database was used to identify 250 cases of sinonasal malignancy in patients aged <20 y (1973-2010). Malignant histology codes were based on the International Classification of Disease for Oncology, third edition coding scheme. Incidence rates were adjusted to the 2000 U.S. population. Survival outcomes were plotted using the Kaplan-Meier method and compared with the log-rank test. All other analyses were performed using standard statistical methods.

RESULTS: Overall incidence was 0.052 per 100,000. Rhabdomyosarcoma had the highest incidence among histologic groups. Regional stage was the most common at diagnosis (59%). Overall survival at 5-y follow-up was 62.5%. Patients in age groups 1-4 and 15-19 y had the worst survival rates, as median survival was 205 and 104 mo, respectively. Distant metastases at the time of diagnosis signified a poor prognosis. These were associated with a 39-mo median survival. Survival improved during the study period, P=0.003. Gender, race, site of lesion, or histology did not appear to affect mortality.

CONCLUSIONS: Sinonasal tumors are rare in children and adolescents. Long-term survival is dependent on age and clinical stage at the time of diagnosis. Cancer-related surgery confers a survival advantage. Gender, race, and histologic type are not associated with mortality.