Epidemiology of pineoblastoma in the United States, 2000-2017

Background Pineoblastoma (PB) is a rare malignant brain tumor originating in the pineal gland. Here, we provide a comprehensive epidemiological analysis of PB in the United States from 2000 to 2017. Methods Data on 1133 patients with PB were acquired from the Central Brain Tumor Registry of the United States, in collaboration with the Centers for Disease Control and Prevention and the National Cancer Institute, from 2000 to 2017. Age-adjusted incidence rates (AAIRs) per 100 000 and incidence rate ratios (IRRs) were reported for age, sex, race, and ethnicity. Using the National Program of Cancer Registries survival database, median survival and hazard ratios (HRs) were evaluated for overall survival from 2001 to 2016. Results Incidence was highest in ages 0-4 years (AAIR: 0.049, 95% CI: 0.042-0.056), decreasing as age increased. Incidence was higher among patients who are Black compared to patients who are White (IRR: 1.71, 95% CI: 1.48-1.98, P < .001), and was impacted by age at diagnosis, with Black-to-White incidence highest in children ages 5-9 years (IRR: 3.43, 95% CI: 2.36-4.94, P < .001). Overall survival was lower for males (HR: 1.39, 95% CI: 1.07-1.79, P = .013). All age groups, excluding those over 40, had improved survival compared to ages 0-4 years. Those who received surgical intervention had better survival compared to those who did not receive surgical treatment. Conclusion PB incidence is highest among children and patients who are Black, and there may be a potential interaction between these factors. Survival is worse among males, young children, and elderly adults, and those who received no surgery. Comprehensive, population-based statistics provide critical information on PB characteristics that could be useful in impacting patient care and prognosis.