Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2019 Aug;144(1):53-63.
doi: 10.1007/s11060-019-03215-x. Epub 2019 Jun 17.

Partnership for defining the impact of 12 selected rare CNS tumors: a report from the CBTRUS and the NCI-CONNECT

Gabrielle Truitt  1   2   3 , Haley Gittleman  1   2   4 , Rebecca Leece  5 , Quinn T Ostrom  1   6 , Carol Kruchko  1 , Terri S Armstrong  7 , Mark R Gilbert  7 , Jill S Barnholtz-Sloan  8   9   10
Affiliations

Partnership for defining the impact of 12 selected rare CNS tumors: a report from the CBTRUS and the NCI-CONNECT

Gabrielle Truitt et al. J Neurooncol. 2019 Aug.

Abstract

Purpose: Population-based cancer statistics, including histology-specific incidence, prevalence, and survival are essential to evaluating the total burden due to disease in a population. The National Cancer Institute's (NCI) Comprehensive Oncology Network Evaluating Rare CNS Tumors (NCI-CONNECT) was developed to better understand tumor biology and patient outcomes for 12 selected brain and other central nervous system (CNS) tumor histologies that are rare in adults to improve approaches to care and treatment. The aim of this study was to determine the incidence, prevalence, and survival of these selected rare histologies.

Methods: Data from the Central Brain Tumor Registry of the United States (CBTRUS) from 2000 to 2014 were used to calculate average annual age-adjusted incidence rates (AAIR) per 100,000 population overall and by sex, race, ethnicity, and age. NCI's Surveillance, Epidemiology and End Results (SEER) data were used to calculate relative survival (RS) estimates. Point prevalence for 2014 was estimated using annual age-specific incidence and survival from CBTRUS and SEER, respectively.

Results: Overall AAIR was 1.47 per 100,000 for all 12 rare histologies combined, with the highest histology-specific incidence in oligodendrogliomas (AAIR = 0.40/100,000). Overall, most histologies were more common in males, adults (age 40 + ), Whites, and non-Hispanics. Ependymomas were the most prevalent histology at 4.11 per 100,000; followed by oligodendrogliomas at 3.68 per 100,000. Relative survival at 1-, 5-, and 10-years was 82.3%, 64.0%, and 55.4%, respectively for all 12 selected brain and other CNS tumor types combined. Ependymomas had the highest RS (1-year = 94.2%, 5-year = 83.9%, 10-year = 78.6%) and gliosarcomas had the lowest relative survival rate (1-year = 42.5%, 5-year = 5.6%, 10-year = 2.9%) at all three time points.

Conclusions: Incidence and prevalence of these rare brain and other CNS tumor histologies have not been previously reported. Along with survival, these data provide a statistical foundation to understand the impact of these cancers and provide important disease-specific data for the design of prospective clinical trials.

Keywords: CNS tumors; Incidence; Prevalence; Rare tumors; Survival.

PubMed Disclaimer

Conflict of interest statement

Conflict of interest There are no conflicts of interest to disclose.

Figures

Fig. 1
Fig. 1
a Annual average age-adjusted incidence rates, brain and other central nervous system (CNS) tumors by selected histology, CBTRUS, 2000–2014; b Tumor distribution, brain and other CNS tumors by selected histology, CBTRUS, 2000–2014; c age-adjusted point prevalence rates, brain and other CNS tumors by selected histology, CBTRUS and SEER, 2014; d 1-, 5-, and 10-year relative survival rates for brain and other CNS tumors by selected histology, SEER 18 Registries, 2000–2014
Fig. 2
Fig. 2
Annual average age-adjusted incidence rates for selected histologies by race and ethnicity, CBTRUS, 2000–2014
Fig. 3
Fig. 3
Annual average age-adjusted incidence rates for selected histologies by age group, CBTRUS, 2000–2014
Fig. 4
Fig. 4
Annual average age-adjusted incidence rates for selected histologies by sex, CBTRUS, 2000–2014
Fig. 5
Fig. 5
Ependymoma frequency by histological type and site, CBTRUS, 2000–2014
See this image and copyright information in PMC

References

    1. Ostrom QT, Gittleman H, Truitt G, Boscia A, Kruchko C, Barnholtz-Sloan JS (2018) CBTRUS statistical report: primary brain and other central nervous system tumors diagnosed in the United States in 2011–2015. Neuro Oncol 20:iv1–iv86. 10.1093/neuonc/noy131 - DOI - PMC - PubMed
    1. Louis DNOH, Wiestler OD, Cavanee WK (eds) (2016) WHO classification of tumours of the central nervous system. International Agency for Research on Cancer, Lyon, France
    1. Surveillance E, and End Results (SEER) Program (2000–2014) SEER*Stat Database: Incidence - SEER 18 Regs Research Data + Hurricane Katrina Impacted Louisiana Cases, Nov 2016 Sub (2000–2014) <Katrina/Rita Population Adjustment> In: National Cancer Institute D, Surveillance Research Program; (ed).
    1. Fay MP (1999) Approximate confidence intervals for rate ratios from directly standardized rates with sparse data. Commun Stat Theory Methods 28:2141–2160. 10.1080/03610929908832411 - DOI
    1. Fay MP, Tiwari RC, Feuer EJ, Zhaohui Z (2006) Estimating average annual percent change for disease rates without assuming constant change. Biometrics 62:847–854 - PubMed

MeSH terms

LinkOut - more resources