Long-term survivors of childhood ewing sarcoma: Report from the childhood cancer survivor study Journal Article


Authors: Ginsberg, J. P.; Goodman, P.; Leisenring, W.; Ness, K. K.; Meyers, P. A.; Wolden, S. L.; Smith, S. M.; Stovall, M.; Hammond, S.; Robison, L. L.; Oeffinger, K. C.
Article Title: Long-term survivors of childhood ewing sarcoma: Report from the childhood cancer survivor study
Abstract: Background The survival of Ewing sarcoma (ES) patients has improved since the 1970s but is associated with considerable future health risks.MethodsThe 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.ResultsCumulative 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).ConclusionLong-term survivors of childhood ES exhibit excess mortality and morbidity. © 2010 The Author.
Keywords: adolescent; adult; cancer survival; child; controlled study; treatment outcome; bone neoplasms; retrospective studies; young adult; major clinical study; united states; logistic models; proportional hazards models; morbidity; risk factors; cancer mortality; childhood cancer; risk assessment; survivors; ewing sarcoma; kaplan-meiers estimate; poisson distribution; chronic disease; disease severity; health status; malignant neoplastic disease; multivariate analysis; neoplasms, second primary; sibling; population surveillance; fertility; sarcoma, ewing's; disability
Journal Title: JNCI: Journal of the National Cancer Institute
Volume: 102
Issue: 16
ISSN: 0027-8874
Publisher: Oxford University Press  
Date Published: 2010-08-18
Start Page: 1272
End Page: 1283
Language: English
DOI: 10.1093/jnci/djq278
PUBMED: 20656964
PROVIDER: scopus
PMCID: PMC2948841
DOI/URL:
Notes: --- - "Cited By (since 1996): 1" - "Export Date: 20 April 2011" - "CODEN: JNCIA" - "Source: Scopus"
Altmetric
Citation Impact
BMJ Impact Analytics
MSK Authors
  1. Suzanne L Wolden
    560 Wolden
  2. Kevin Oeffinger
    297 Oeffinger
  3. Paul Meyers
    311 Meyers