Descriptive epidemiology of selected olfactory tumors.

Olfactory tumors, especially olfactory neuroblastomas (ON) and carcinomas with neuroendocrine differentiation (CND), are extremely rare, and little descriptive epidemiologic information is available. The objective of this study was to more fully describe selected olfactory tumors using a large population-based cancer incidence database. The Surveillance, Epidemiology and End Results (SEER) 9 registries limited-use data were reviewed from 1973 to 2006 for selected nasal cavity (C30.0) and accessory sinus (C31.0-31.9) tumors. Frequencies, incidence rates, and relative survival rates were estimated using SEER*Stat, v6.5.2. The majority of cases were squamous cell carcinoma (SCC), while the incidence of ON was greater than CND. For ON, the incidence was highest in the 60-79 year age group, while for SCC, the incidence was highest in the 80+ year age group. For CND, the incidence leveled off in the oldest age groups. Survival rates were highest for ON (>70% alive at 5 years after diagnosis) and poorest for CND (44% alive at 5 years). Adjuvant radiation therapy did not improve survival over surgery alone in ON. In SCC, survival was worse in patients who received adjuvant radiation compared to patients who had surgery alone. Our analysis confirms some previously published information, and adds new information about the incidence and demographics of ON and CND. In addition, our analysis documents the lack of benefit of adjuvant radiation in ON. It is not feasible to conduct prospective trials in patients with these rare diseases, and the importance of registry data in learning about olfactory tumors is emphasized.