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Comparative Study
. 2021 Sep 1;149(5):1054-1066.
doi: 10.1002/ijc.33674. Epub 2021 May 17.

Comparative international incidence of Ewing sarcoma 1988 to 2012

Affiliations
Comparative Study

Comparative international incidence of Ewing sarcoma 1988 to 2012

Logan G Spector et al. Int J Cancer. .

Abstract

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.

Keywords: Ewing sarcoma; incidence; international.

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Conflict of interest statement

Conflict of Interest

The authors have no conflict of interest relevant to this article to disclose.

Figures

Figure 1:
Figure 1:
Incidence rate (IR) per million of Ewing Sarcoma (ES) by time-period for each age group in each geographic region, Cancer Incidence in Five Continents (CI5) 1988–2012. *No data were available for the Caribbean region in the 1988–1992 time-period.
Figure 1:
Figure 1:
Incidence rate (IR) per million of Ewing Sarcoma (ES) by time-period for each age group in each geographic region, Cancer Incidence in Five Continents (CI5) 1988–2012. *No data were available for the Caribbean region in the 1988–1992 time-period.
Figure 2:
Figure 2:
Male to female Ewing sarcoma (ES) incidence rate ratios (IRR) and 95% confidence intervals (CI) by geographic region in 10–14 and 15–19-year-old age groups, Cancer Incidence in Five Continents (CI5) 2003–2012.
Figure 3:
Figure 3:
Ewing Sarcoma (ES) incidence rates (IR) per million among 10–19-year-olds among countries, Cancer Incidence in Five Continents (CI5) 2003–2012.
Figure 4:
Figure 4:
Incidence rate (IR) per million and 95% confidence intervals (CI) of Ewing Sarcoma (ES) among 10–19-year-olds by country and predominant ancestry, Cancer Incidence in Five Continents (CI5) 2003–2012. *Groups with a non-classifiable ancestry were not included.
Figure 5:
Figure 5:
(A) Odds ratios (OR) and 95% confidence intervals (CI) for SNPs identified from published GWAS literature in Ewing Sarcoma (ES), and (B) SNP risk allele frequencies from 1000 Genomes in genes among European, Hispanic, East Asian and African populations.

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