Work

Exth-61. Partnership for Defining the Impact of 12 Select Rare Cns Tumors: a Report From Cbtrus and the Nci-connect

Year: 2018

Type: conference-abstract

Abstract: Measuring tumor-specific incidence, prevalence, and survival is essential to evaluate the contribution of specific tumor-types to overall cancer burden in the United States population. Central nervous system tumors are rare, with specific tumor types rarer than others. NCI-Connect was developed to understand tumor biology, patient outcomes, and develop standards of care for 12 select rare CNS Tumors. The aim of this study was to determine the incidence, prevalence, and survival of specific rare brain tumors by histology, age, race, and sex in this subset. Data derived from CBTRUS was used to examine age-adjusted incidence rate (AAIR) per 100,000 and data derived from SEER was used to examine prevalence and survival statistics from 2000–2014 (all with 95% confidence intervals). The data was then used to compare specific rare brain tumors overall and by sex, age group, race, and ethnicity. The total AAIR was 6.57/100,000 for these specific brain tumor types combined; with the highest AAIR at 0.4/100,000 for oligodendroglioma. Overall, the specified tumors were most incident in males, adults (age 40+), white individuals, and non-Hispanic individuals. Ependymomas were most prevalent at 4.11 cases per 100,000; followed by brain stem and midline gliomas at 1.98 cases per 100,000. Relative survival at 1-, 5-, and 10-years was 59.7%, 33.3%, and 27.7% respectively for all subtypes. Ependymomas had the highest relative survival rate (94.2%, 83.9%, and 78.6%) and gliosarcomas had the lowest relative survival rate (42.5%, 5.6%, 2.9%) at all three time points. Overall incidence and prevalence of these brain tumors was very low and varied considerably by each tumor type. Survival also varied by tumor type. The comparison of incidence, prevalence, and survival of rare brain and CNS tumors is critical for measuring the clinical impact of these diseases and for determining future research and clinical care.

Source: Neuro-Oncology

DOI: 10.1093/neuonc/noy148.408