Comparative international incidence of Ewing sarcoma 1988 to 2012

• Published in: International journal of cancer 2021-09, Vol.149 (5), p.1054-1066
• Main Authors: Spector, Logan G., Hubbard, Aubrey K., Diessner, Brandon J., Machiela, Mitchell J., Webber, Beau R., Schiffman, Joshua D.
• Format: Article
• Language: English
• Subjects: Bone tumors; Cancer; Children; Epidemiology; Ewing sarcoma; Ewing's sarcoma; Ewings sarcoma; incidence; international; Medical research; Risk factors; Statistical analysis; Subpopulations
• ISSN: 0020-7136; 1097-0215
• DOI: 10.1002/ijc.33674

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. What's new? Racial and ethnic disparities in Ewing sarcoma incidence are observed in the United States and elsewhere, but international incidence rates have not been systematically evaluated recently. Here, the authors used data from the Cancer Incidence in Five Continents database to describe trends in Ewing sarcoma incidence across time, geographic regions, and race/ethnicity. The results confirm that Ewing sarcoma incidence rates vary substantially across populations and reveal rising rates that are not consistent across geographic regions or age groups. The study highlights a need for future research on the association of genomic ancestry with Ewing sarcoma risk.