Survivors of childhood Ewing sarcoma at increased risk for mortality, secondary cancers

Ginsberg JP. J Natl Cancer Inst. 2010;102:1-12.

Survivors of childhood Ewing sarcoma are at increased risk for mortality, are more likely to develop subsequent malignant neoplasms and are more likely than their siblings to have severe, life-threatening, or disabling chronic health conditions, according to new data from the Childhood Cancer Survivor Study.

For the study, researchers at 26 institutions in the U.S. and Canada surveyed 403 survivors treated in the 1970s and 1980s in comparison with 3,899 sibling controls. Mean follow-up from diagnosis to last contact was 23 years for surviving participants. Time to death ranged from 5 to 28 years.

Twenty years after diagnosis, OS was 78.5% (95% CI, 74.9-81.8). Among patients who survived at least 5 years from diagnosis, 75% were still alive after 25 years, translating into a 25% cumulative mortality (95% CI, 21.1-28.9).

Including recurrence, the overall all-cause standardized mortality ratio was 13.3 (95% CI, 11.2-15.8). The ration was 23.1 for women (95% CI, 17.6-29.7) and 10 for men (95% CI, 7.9-12.5).

Progression or recurrence of the primary Ewing sarcoma accounted for 60.3% of deaths. Subsequent malignant neoplasms were the second leading cause, accounting for 19 deaths for a standardized mortality ratio of 20 (95% CI, 12.0-31.6).

Further, the cumulative mortality due to cardiac and pulmonary disease potentially attributable to treatment and to malignant neoplasms not related to the primary tumor was 8.3% (95% CI, 5.4-11.3).

“In addition to increased mortality following Ewing sarcoma, survivors are at substantially higher risk of morbidity because of second malignancies, chronic health conditions and functional impairment, all of which require extensive clinical management,” the researchers wrote. “Enrollment and long-term follow-up in late effect programs is clearly indicated in this cohort.”

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