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

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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.

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Funding

Funding for CBTRUS has been provided by the Centers for Disease Control and Prevention (CDC) under Contract No. 2016-M-9030, the American Brain Tumor Association, The Sontag Foundation, Novocure, AbbVie, the Musella Foundation, National Brain Tumor Society, the Zelda Dorin Tetenbaum Memorial Fund, the National Cancer Institute (NCI) under Contract No. HHSN261201800176P, the Uncle Kory Foundation, and from private and in kind donations. The findings and conclusions in this report are those of the authors and do not necessarily represent the official position of the CDC or NCI. QTO is supported by a Research Training Grant from the Cancer Prevention and Research Institute of Texas (CPRIT; RP160097T).

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Authors and Affiliations

  1. Central Brain Tumor Registry of the United States, Hinsdale, IL, USA

    Gabrielle Truitt, Haley Gittleman, Quinn T. Ostrom, Carol Kruchko & Jill S. Barnholtz-Sloan

  2. Case Comprehensive Cancer Center, Cleveland, OH, USA

    Gabrielle Truitt, Haley Gittleman & Jill S. Barnholtz-Sloan

  3. Department of Bioethics, Case Western Reserve University School of Medicine, Cleveland, OH, USA

    Gabrielle Truitt

  4. Department of Population and Quantitative Health Sciences, Case Western Reserve University School of Medicine, Cleveland, OH, USA

    Haley Gittleman & Jill S. Barnholtz-Sloan

  5. Case Western Reserve University School of Medicine, Cleveland, OH, USA

    Rebecca Leece

  6. Department of Medicine, Section of Epidemiology and Population Sciences, Dan L. Duncan Comprehensive Cancer Center, Baylor College of Medicine, Houston, TX, USA

    Quinn T. Ostrom

  7. Neuro Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA

    Terri S. Armstrong & Mark R. Gilbert

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  1. Gabrielle Truitt
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  2. Haley Gittleman
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  3. Rebecca Leece
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Correspondence to Jill S. Barnholtz-Sloan.

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This article does not contain any studies with human participants or animals performed by any of the authors. This study was approved as an exempt study by the University Hospitals Cleveland Medical Center Institutional Review Board.

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This was a retrospective study using de-identified national cancer registries. Thus, formal consent was not required.

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Truitt, G., Gittleman, H., Leece, R. et al. Partnership for defining the impact of 12 selected rare CNS tumors: a report from the CBTRUS and the NCI-CONNECT. J Neurooncol 144, 53–63 (2019). https://doi.org/10.1007/s11060-019-03215-x

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