Longitudinal follow-up of adult survivors of Ewing sarcoma: A report from the Childhood Cancer Survivor Study Journal Article


Authors: Marina, N. M.; Liu, Q.; Donaldson, S. S.; Sklar, C. A.; Armstrong, G. T.; Oeffinger, K. C.; Leisenring, W. M.; Ginsberg, J. P.; Henderson, T. O.; Neglia, J. P.; Stovall, M. A.; Yasui, Y.; Randall, R. L.; Geller, D. S.; Robison, L. L.; Ness, K. K.
Article Title: Longitudinal follow-up of adult survivors of Ewing sarcoma: A report from the Childhood Cancer Survivor Study
Abstract: BACKGROUND: Ewing sarcoma survivors (ESSs) are at increased risk for treatment-related complications. The incidence of treatment-related morbidity and late mortality with aging is unknown. METHODS: This study reports survival probabilities, estimated with the Kaplan-Meier method, and the cumulative incidence of cause-specific mortality and chronic conditions among ESSs in the Childhood Cancer Survivor Study who were treated between 1970 and 1986. Piecewise exponential models were used to estimate relative rates (RRs) and 95% confidence intervals (CIs) for these outcomes. Chronic conditions were graded with the Common Terminology Criteria for Adverse Events (version 4.03). RESULTS: Among 404 5-year ESSs (median age at last follow-up, 34.8 years; range, 9.1-54.8 years), the 35-year survival rate was 70% (95% CI, 66%-74%). Late recurrence (cumulative incidence at 35 years, 15.1%) was the most common cause of death, and it was followed by treatment-related causes (11.2%). There were 53 patients with subsequent neoplasms (SNs; cumulative incidence at 35 years, 24.0%), and 38 were malignant (14.3% at 35 years). The standardized incidence ratios were 377.1 (95% CI, 172.1-715.9) for osteosarcoma, 28.9 (95% CI, 3.2-104.2) for acute myeloid leukemia, 14.9 (95% CI, 7.9-25.5) for breast cancer, and 13.1 (95% CI, 4.8-28.5) for thyroid cancer. Rates of chronic conditions were highest for musculoskeletal (RR, 18.1; 95% CI, 12.8-25.7) and cardiac complications (RR, 1.8; 95% CI, 1.4-2.3). Thirty-five years after the diagnosis, the cumulative incidences of any chronic conditions and 2 or more chronic conditions were 84.6% (95% CI, 80.4%-88.8%) and 73.8% (95% CI, 67.8%-79.9%), respectively. CONCLUSIONS: With extended follow-up, ESSs' risk for late mortality and SNs does not plateau. Treatment-related chronic conditions develop years after therapy, and this supports the need for lifelong follow-up. Cancer 2017;123:2551–60. © 2017 American Cancer Society. © 2017 American Cancer Society
Keywords: ewing sarcoma; childhood cancer survivors; chronic health conditions; late mortality; treatment-related complications
Journal Title: Cancer
Volume: 123
Issue: 13
ISSN: 0008-543X
Publisher: Wiley Blackwell  
Date Published: 2017-07-01
Start Page: 2551
End Page: 2560
Language: English
DOI: 10.1002/cncr.30627
PROVIDER: scopus
PMCID: PMC5474122
PUBMED: 28222219
DOI/URL:
Notes: Article -- Export Date: 1 August 2017 -- Source: Scopus
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  1. Charles A Sklar
    322 Sklar
  2. Kevin Oeffinger
    296 Oeffinger