Esthesioneuroblastoma: Descriptive Epidemiology from the Central Brain Tumor Registry of the United States

Introduction: Esthesioneuroblastoma, also referred to as olfactory neuroblastoma, is a rare malignant neoplasm arising from olfactory neuroepithelium. Aiming to provide a comprehensive epidemiologic analysis of esthesioneuroblastomas, this study draws from the Central Brain Tumor Registry of the United States (CBTRUS), which contains population-based incidence data covering 99% of the United States population from 1994 to 2014.

Methods: CBTRUS was queried for esthesioneuroblastomas based on ICD-O-3 codes. Age-adjusted incidence per 100,000 persons was calculated and stratified by race, sex, age, lesion location, and diagnostic method. Joinpoint Regression Program was used to calculate annual percentage change in incidence rates over time.

Results: A total of 2,103 esthesioneuroblastomas were diagnosed in the United States from 2001 to 2014, averaging ∼150 cases annually. The most common location was the nasal cavity (1709 cases). Those not located in the nasal cavity were found in the ethmoid (182 cases), sphenoid (50 cases), maxillary (39 cases), or accessory sinuses (68 cases). Overall age-adjusted rate was 0.0477 per 100,000 (95% CI, 0.0457–0.0498). There was no statistically significant change in annual rates from 2001 to 2014. A peak rate of 0.1331 per 100,000 (95% CI, 0.1118–0.1571) occurred in patients aged 75–79 years. The rate in males (0.0587 per 100,000; 95% CI, 0.0555–0.0622) was significantly higher (p <  0.05) than in females (0.0013 per 100,000; 95% CI, 0.0354–0.0406). The rate was highest in non-Hispanic white patients at 0.0495 per 100,000 (95% CI, 0.0472–0.0519). The rate ratio of esthesioneuroblastoma in black patients to non-Hispanic white patients was 0.6452 (p <  0.05). There were not enough American Indian/Alaska Native patients to form definitive conclusions.

Conclusion: The incidence of esthesioneuroblastomas was higher in males relative to females, and in non-Hispanic white patients relative to black patients. Incidence peaked in patients aged 75 to 79 years. This study represents the most comprehensive epidemiological analysis of esthesioneuroblastoma incidence rate in the United States to date.