Long-term Survivors of Childhood Ewing Sarcoma: Report From the Childhood Cancer Survivor Study

• Published in: JNCI : Journal of the National Cancer Institute 2010-08, Vol.102 (16), p.1272-1283
• Main Authors: Ginsberg, Jill P., Goodman, Pamela, Leisenring, Wendy, Ness, Kirsten K., Meyers, Paul A., Wolden, Suzanne L., Smith, Stephanie M., Stovall, Marilyn, Hammond, Sue, Robison, Leslie L., Oeffinger, Kevin C.
• Format: Article
• Language: English
• Subjects: Adolescent; Adult; Biological and medical sciences; Bone Neoplasms - mortality; Cancer; Child; Chronic Disease - epidemiology; Clinical outcomes; Diseases of the osteoarticular system; Female; Fertility; Health Status; Humans; Kaplan-Meier Estimate; Logistic Models; Male; Medical sciences; Morbidity; Multivariate Analysis; Neoplasms, Second Primary - epidemiology; Poisson Distribution; Population Surveillance; Proportional Hazards Models; Retrospective Studies; Risk Factors; Sarcoma, Ewing - mortality; Studies; Survivor; Survivors - statistics & numerical data; Treatment Outcome; Tumors; Tumors of striated muscle and skeleton; United States - epidemiology; Young Adult
• ISSN: 0027-8874; 1460-2105
• DOI: 10.1093/jnci/djq278

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 (≥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.