The risk for late mortality and subsequent neoplasms does not plateau among survivors of childhood Ewing sarcoma, suggesting the need for lifelong follow-up, according to a study published in Cancer.1

Previous reports demonstrated that Ewing sarcoma survivors have an increased risk for developing treatment-related complications, including anthracycline-induced cardiomyopathy and subsequent neoplasms. There are, however, limited long-term follow-up data crucial to informing survivorship care.

To evaluate the incidence of treatment-related morbidity and late mortality in adult survivors of Ewing sarcoma during childhood, investigators retrospectively analyzed data from 404 participants of the Childhood Cancer Survivor Study who were treated between 1970 and 1986.

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Seventy percent (95% CI, 66-74) of survivors were alive after 35 years of follow-up. Late recurrence occurred in 15.1% of patients and was the most common cause of death, followed by treatment-related causes in 11.2%.

Nearly a quarter (24.0%) of survivors developed subsequent neoplasms, including 14.3% who developed a malignant neoplasm. Breast cancer was the most frequent secondary malignant neoplasm, though some patients also developed osteosarcoma, thyroid cancer, and acute myeloid leukemia.

Nearly all survivors who developed secondary malignant neoplasms previously received alkylating agents, anthracyclines, and radiotherapy, but only receipt of chest radiotherapy was associated with the risk of secondary malignancy.

Investigators found that 84.6% (95% CI, 80.4-88.8) and 73.8% (95% CI, 67.8-79.9) of survivors had developed any number of chronic conditions and 2 or more chronic conditions, respectively, during follow-up. The most common chronic conditions experienced by survivors were musculoskeletal and cardiac complications.

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Given that adult survivors of Ewing sarcoma have a high incidence of recurrent disease leading to death and secondary malignant neoplasms, the findings suggest that long-term follow-up is needed for this population.


  1. Marina NM, Liu Q, Donaldson SS, et al. Longitudinal follow-up of adult survivors of Ewing sarcoma: A report from the Childhood Cancer Survivor Study. Cancer. 2017 Feb 21. doi: 10.1002/cncr.30627 [Epub ahead of print]