Primary brain and other central nervous system tumors in Appalachia: regional differences in incidence, mortality, and survival

  • Quinn T. Ostrom
  • Haley Gittleman
  • Carol Kruchko
  • Jill S. Barnholtz-SloanEmail author
Laboratory Investigation



The Appalachian region is a large geographic and economic area, representing 7.69% of the United States (US). This region is more rural, whiter, older, and has a higher level of poverty as compared to the rest of the US. Limited research has been done on primary brain and other central nervous system tumors (PBT) epidemiology in this region. In this analysis we characterize incidence, mortality, and survival patterns.


Data from 2006 to 2015 were obtained from the central brain tumor registry of the US (provided by CDC and NCI). Appalachian counties were categorized using the Appalachia Regional Council scheme. Overall and histology-specific age-adjusted incidence and mortality rates per 100,000 population were generated. 1-, 5-, and 10-year relative survival (RS) was estimated using CDC national program of cancer registry data from 2001 to 2014.


Overall PBT incidence within Appalachia was 22.62 per 100,000, which is not significantly different from the non-Appalachian US (22.77/100,000, p = 0.1189). Malignant incidence was 5% higher in Appalachia (7.55/100,000 vs. 7.23/100,000, p < 0.0001), while non-malignant incidence was 3% lower (15.07/100,000 vs. 15.54/100,000, p < 0.0001). 5-year RS for malignant PBT was lower (31.4% vs. 36.0%), and mortality due to malignant PBT was higher in Appalachia (4.86/100,000 vs. 4.34/100,000, p < 0.0001).


Appalachia has increased malignant and decreased non-malignant PBT incidence, and poorer survival outcomes for malignant PBT compared to the non-Appalachian US.


Brain and CNS tumors Incidence Relative survival Mortality Appalachia 



This work was previously presented at the 2018 annual meeting of the Society for Neuro-Oncology.

Author Contributions

Conceptualization: QTO. Formal analysis: QTO. Data curation: QTO, HG. Writing—original draft: QTO. Writing - review and editing: QTO, HG, CK, JSB. Funding acquisition: CK, JSB.


QTO is supported by a Research Training Grant from the Cancer Prevention and Research Institute of Texas (CPRIT; RP160097T). Funding for CBTRUS was 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, and the National Cancer Institute (NCI) under contract No. HHSN261201800176P, as well as private and in kind donations. Contents are solely the responsibility of the authors and do not necessarily reflect the official views of the CDC or the NCI.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This study was approved by the Institutional Review Board at University Hospitals Cleveland Medical Center.

Informed consent

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

Supplementary material

11060_2018_3073_MOESM1_ESM.docx (1.1 mb)
Supplementary material 1 (DOCX 1124 KB)


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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Central Brain Tumor Registry of the United StatesHinsdaleUSA
  2. 2.Department of Medicine, Section of Epidemiology and Population Sciences, Dan L. Duncan Comprehensive Cancer CenterBaylor College of MedicineHoustonUSA
  3. 3.Department of Population and Quantitative Health Sciences, Case Comprehensive Cancer Center,Case Western Reserve University School of MedicineClevelandUSA

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