Comparative international incidence of Ewing sarcoma 1988 to 2012

Logan G. Spector, Aubrey K Hubbard, Brandon J. Diessner, Mitchell J. Machiela, Beau R. Webber, Joshua D. Schiffman

Research output: Contribution to journalArticlepeer-review


Ewing sarcoma (ES) is the second most common primary bone tumor in children and adolescents. There are few known epidemiological or genetic risk factors for ES. Numerous reports describe incidence rates and trends within the United States, but international comparisons are sparse. We used the Cancer Incidence in Five Continents (CI5) data to estimate age standardized incidence rates (ASRs; cases per million) and 95% confidence intervals (95% CIs), male-to-female incidence rate ratios (IRRs; 95% CI), and the average annual percent change in incidence (AAPC; 95% CI) for ES by geographic region for children and adults aged 0 to 49 years. We also estimated the ASR for each country or country subpopulation among the 10- to 19-year-old age range; capturing the peak incidence of ES. In total, 15 874 ES cases ages 0 to 49 were reported in the CI5 series between 1988 and 2012. AAPC estimates varied by age group and geographic region. Most of the statistically significant AAPCs showed an increased incidence over time; the only statistically significant decreases in incidence were observed among 20- to 29-year-olds and 30- to 39-year-olds in Southern Asia at −1.93% and −1.67%. When categorized by predominant ancestry, we observed countries and subpopulations with predominately African, East Asian, and Southeast Asian ancestry had the lowest incidence rates, whereas Pacific Islanders and populations with predominantly European and North African/Middle Eastern ancestry had the highest. An excess incidence in males was observed in most regions. Our results highlight substantial variation in ES incidence across geographic populations, reflecting potential ancestral influence on disease risk.

Original languageEnglish (US)
Pages (from-to)1054-1066
Number of pages13
JournalInternational Journal of Cancer
Issue number5
StatePublished - Sep 1 2021

Bibliographical note

Funding Information:
Research reported in this publication was supported by NIH grant P30CA077598 utilizing the Biostatistics and Bioinformatics Core shared resource of the Masonic Cancer Center, University of Minnesota and by the National Center for Advancing Translational Sciences of the National Institutes of Health Award Number UL1‐TR002494. The content is solely the responsibility of the authors and does not necessarily represent the official views of the National Institutes of Health. This work was also supported by grants from the National Institutes of Health (T32 CA099936 to A.K. Hubbard and T32 AR050938 to B. J. Diessner) as well as the Intramural Research Program of the National Cancer Institute.

Publisher Copyright:
© 2021 UICC.


  • Ewing sarcoma
  • incidence
  • international

PubMed: MeSH publication types

  • Comparative Study
  • Journal Article
  • Research Support, N.I.H., Extramural


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