Epidemiology of vestibular schwannoma in the United States, 2004–2016

BACKGROUND: Vestibular schwannomas (VS) are nonmalignant tumors of the eighth cranial nerve and are the most common nonmalignant nerve sheath tumor. This study provides the most comprehensive and current analysis of VS epidemiology in the United States. METHODS: Incidence data were obtained from the Central Brain Tumor Registry of the United States, from 2004 to 2016 for VS. Age-adjusted incidence rates (AAIRs), rate ratios (AAIRRs), and prevalence ratios (AAPRs) per 100 000 were analyzed by age, sex, race and ethnicity, and laterality. Additional analyses were performed to assess differences in treatment, laterality, and diagnostic confirmation. RESULTS: Incidence of VS was highest among adults (aged 65–74 years, AAIR: 3.18, 95% confidence interval [CI]: 3.15–3.25). However, there was a much higher distribution of bilateral tumors compared to unilateral in children aged 0–19 years (28.5% vs 1.0%, P < .001). VS incidence was highest among white non-Hispanics (AAIR:1.30, 95% CI: 1.29–1. 31) and lowest among black non-Hispanics. Incidence of radiographically confirmed VS increased from 2004 to 2016 (annual percent change: 1.64, 95% CI: 0.15–3.16, P = .03). For treatment, 40.1% received surgery, while only 23.7% received radiation. There were an estimated 44 762 prevalent cases of VS in 2016 (AAPR: 12.17, 95% CI: 12.06–12.29). CONCLUSIONS: VS incidence and prevalence are highest among adults and white non-Hispanics. Bilateral VS was more common among children. There was an increase of radiographically confirmed VS over time. A higher proportion of patients received surgical treatment than radiotherapy. Population-based statistics provide healthcare professionals with vital information regarding disease burden and help improve patient care.