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Cancer. 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.

Author information

1
Department of Pediatrics, Stanford University and Lucile Packard Children's Hospital, Palo Alto, California.
2
School of Public Health, University of Alberta, Edmonton, Alberta, Canada.
3
Depratment of Radiation Oncology, Stanford University, Stanford, California.
4
Department of Medicine, Memorial Sloan Kettering Cancer Center, New York, New York.
5
Department of Epidemiology and Cancer Control, St. Jude Children's Research Hospital, Memphis, Tennessee.
6
Seattle Children's Hospital, Clinical Statistics, Seattle, Washington.
7
Department of Pediatric Oncology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
8
University of Chicago, Comprehensive Cancer Center, Pediatric Oncology, Chicago, Illinois.
9
University of Minnesota/Masonic Cancer Center, Pediatrics, Minneapolis, Minnesota, Minnesota.
10
The University of Texas MD Anderson Cancer Center, Houston, Texas.
11
Department of Orthopedic Surgery, Primary Children's Hospital and Huntsman Cancer Institute, University of Utah, Salt Lake City, Utah.
12
Montefiore Medical Center-Moses Campus and Orthopedic Surgery, Children's Hospital at Montefiore, Bronx, New York.

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

PMID:
28222219
PMCID:
PMC5474122
DOI:
10.1002/cncr.30627
[Indexed for MEDLINE]
Free PMC Article

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