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. 2010 Aug 18;102(16):1272-83.
doi: 10.1093/jnci/djq278. Epub 2010 Jul 23.

Long-term survivors of childhood Ewing sarcoma: report from the childhood cancer survivor study

Jill P Ginsberg  1 , Pamela Goodman, Wendy Leisenring, Kirsten K Ness, Paul A Meyers, Suzanne L Wolden, Stephanie M Smith, Marilyn Stovall, Sue Hammond, Leslie L Robison, Kevin C Oeffinger
Affiliations

Long-term survivors of childhood Ewing sarcoma: report from the childhood cancer survivor study

Jill P Ginsberg et al. J Natl Cancer Inst. .

Abstract

Background: The survival of Ewing sarcoma (ES) patients has improved since the 1970s but is associated with considerable future health risks.

Methods: The study population consisted of long-term (> or =5-year) survivors of childhood ES diagnosed before age 21 from 1970 to 1986. Cause-specific mortality was evaluated in eligible survivors (n = 568), and subsequent malignant neoplasms, chronic health conditions, infertility, and health status were evaluated in the subset participating in the Childhood Cancer Survivor Study (n = 403). Outcomes were compared with the US population and sibling control subjects (n = 3899). Logistic, Poisson, or Cox proportional hazards models, with adjustments for sex, age, race/ethnicity, and potential intrafamily correlation, were used. Statistical tests were two-sided.

Results: Cumulative mortality of ES survivors was 25.0% (95% confidence interval [CI] = 21.1 to 28.9) 25 years after diagnosis. The all-cause standardized mortality ratio was 13.3 (95% CI = 11.2 to 15.8) overall, 23.1 (95% CI = 17.6 to 29.7) for women, and 10.0 (95% CI = 7.9 to 12.5) for men. The nonrecurrence-progression non-external cause standardized mortality ratio (subsequent non-ES malignant neoplasms and cardiac and pulmonary causes potentially attributable to ES treatment) was 8.7 (95% CI = 6.2 to 12.0). Twenty-five years after ES diagnosis, cumulative incidence of subsequent malignant neoplasms, excluding nonmelanoma skin cancers, was 9.0% (95% CI = 5.8 to 12.2). Compared with siblings, survivors had an increased risk of severe, life-threatening, or disabling chronic health conditions (relative risk = 6.0, 95% CI = 4.1 to 9.0). Survivors had lower fertility rates (women: P = .005; men: P < .001) and higher rates of moderate to extreme adverse health status (P < .001).

Conclusion: Long-term survivors of childhood ES exhibit excess mortality and morbidity.

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Figures

Figure 1
Figure 1
Flow diagram of Ewing sarcoma (ES) survivors and siblings in the Childhood Cancer Survivor Study (CCSS).
Figure 2
Figure 2
Overall survival from Kaplan–Meier estimates, with 95% confidence intervals among 5-year survivors of Ewing sarcoma compared with expected survival using age, sex, and year standardized to the US population.
Figure 3
Figure 3
Cumulative incidence of mortality attributable to nonrecurrence-progression non-external causes (subsequent non-Ewing sarcoma (ES) malignant neoplasms and cardiac and pulmonary causes) among 5-year survivors of ES, treating death from other causes as competing risk events. A) Mortality for entire ES cohort. B) Mortality by radiation therapy (RT).
Figure 4
Figure 4
Subsequent malignant neoplasms (SMN) and chronic health conditions among 5-year survivors of Ewing sarcoma. A) Cumulative incidence with 95% confidence intervals of SMN (excluding nonmelanoma skin cancer) with death as a competing risk. B) Cumulative incidence with 95% confidence intervals of chronic health conditions according to Common Terminology Criteria for Adverse Events, version 3, severity grades (grades 1–5 vs grades 3–5).
See this image and copyright information in PMC

References

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