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Descriptive epidemiology of chordomas in the United States

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Abstract

Purpose

Chordomas account for 1% to 4% of all bone malignancies and 0.5% of all primary intracranial central nervous system tumors. Prior epidemiologic literature is based on limited population data. The purpose of this study is to provide the largest and most inclusive population based study of the descriptive epidemiology of chordomas.

Methods

The Centers for Disease Control and Prevention and National Program of Cancer Registries were queried for chordoma in all locations. Age-adjusted incidence per 100,000 persons was calculated by age, sex, race, and ethnicity. Annual percentage change was calculated using Joinpoint.

Results

From 2004 to 2014, a total of 3670 chordomas were diagnosed in the US. The most common location was cranial (38.7%), followed by sacral (34.3%) and spinal (27.0%). The average age-adjusted incidence rate was 0.088 per 100,000 persons per year (95% CI 0.086–0.091), with an annual percentage change of 1.29% (95% CI 0.31–2.28%). For all chordomas, the incidence peaks in the 75–84 year age group. The male-to-female incidence rate ratio is 1.54 (p < 0.001). American Indian/Alaskan Native and Black patients had a statistically lower incidence rate than White and Asian/Pacific Islander patients.

Conclusion

Approximately 0.088 chordomas per 100,000 persons are newly diagnosed in the US each year, with cranial location being the most common, followed by sacral and spinal. Incidence increases with age, and men are at a significantly higher risk than women. This investigation represents the largest population-based epidemiologic study of chordomas in the US.

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Data availability

The authors confirm that the data supporting the findings of this study are available within the article and from the databases mentioned within.

Code availability

The code that supports the findings of this study are available from the corresponding author, upon reasonable request.

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Authors and Affiliations

  1. Section of Neurosurgery, University of Chicago, Chicago, IL, USA

    Paramita Das

  2. Department of Neurosurgery, Neurological Institute, Cleveland Clinic, Cleveland, OH, USA

    Pranay Soni, Ghaith Habboub, Pablo F. Recinos & Varun R. Kshettry

  3. Rose Ella Burkhardt Brain Tumor & Neuro-Oncology Center, Cleveland Clinic, Cleveland, OH, USA

    Pranay Soni, Pablo F. Recinos & Varun R. Kshettry

  4. Department of Neurosurgery, University of Michigan, Ann Arbor, MI, USA

    Jaes Jones

  5. Case Comprehensive Cancer Center, Case Western Reserve University Medical School, Cleveland, OH, USA

    Jill S. Barnholtz-Sloan

  6. Skull Base & Cerebrovascular Surgery, Department of Neurosurgery, Cleveland Clinic, 9500 Euclid Avenue, CA5-86, Cleveland, OH, 44195, USA

    Varun R. Kshettry

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  1. Paramita Das
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Correspondence to Varun R. Kshettry.

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Varun R. Kshettry is a consultant for Integra LifeSciences.

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Das, P., Soni, P., Jones, J. et al. Descriptive epidemiology of chordomas in the United States. J Neurooncol 148, 173–178 (2020). https://doi.org/10.1007/s11060-020-03511-x

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  • DOI: https://doi.org/10.1007/s11060-020-03511-x

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