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. 2017 Jul 1;123(13):2551-2560.
doi: 10.1002/cncr.30627. Epub 2017 Feb 21.

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

Neyssa M Marina  1 , Qi Liu  2 , Sarah S Donaldson  3 , Charles A Sklar  4 , Gregory T Armstrong  5 , Kevin C Oeffinger  4 , Wendy M Leisenring  6 , Jill P Ginsberg  7 , Tara O Henderson  8 , Joseph P Neglia  9 , Marilyn A Stovall  10 , Yutaka Yasui  5 , R Lor Randall  11 , David S Geller  12 , Leslie L Robison  5 , Kirsten K Ness  5
Affiliations

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

Neyssa M Marina et al. Cancer. .

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.

Keywords: Ewing sarcoma; childhood cancer survivors; chronic health conditions; late mortality; treatment-related complications.

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Conflict of interest statement

Conflict of interest disclosures: Dr. Marina consultant Nekktar and Dr. Randall consultant Zimmer Biomet

Figures

Figure 1
Figure 1. Cumulative incidence of second neoplasms (malignant and non-malignant)
Figure 2
Figure 2. Cumulative incidence of musculoskeletal and cardiac complications
See this image and copyright information in PMC

Comment in

  • Second malignancies in Ewing sarcoma survivors.
    Lin AY, Hall ET. Lin AY, et al. Cancer. 2017 Oct 15;123(20):4075. doi: 10.1002/cncr.30922. Epub 2017 Aug 24. Cancer. 2017. PMID: 28837218 No abstract available.
  • Reply to Second malignancies in Ewing sarcoma survivors.
    Marina NM, Liu Q, Donaldson SS, Sklar CA, Armstrong GT, Oeffinger KC, Leisenring WM, Ginsberg JP, Henderson TO, Neglia JP, Stovall MA, Yasui Y, Randall RL, Geller DS, Robison LL, Ness KK. Marina NM, et al. Cancer. 2017 Oct 15;123(20):4075-4076. doi: 10.1002/cncr.30921. Epub 2017 Aug 24. Cancer. 2017. PMID: 28837220 Free PMC article. No abstract available.

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