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Long-term Ewing’s sarcoma survivors still face high mortality risk
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Most late deaths in 5-year survivors of Ewing’s sarcoma are due to their disease, according to findings from a retrospective cohort study.
The late risk for death 5 and 15 years after diagnosis of Ewing’s sarcoma appeared even higher in older patients, black patients and men.
Steven G. DuBois, MD
Steven G. DuBois, MD, director of experimental therapeutics at Dana-Farber Cancer Institute and a member of the faculty of pediatrics at Harvard Medical School in Boston, and colleagues sought to describe the clinical features and outcomes of patients with Ewing’s sarcoma who have survived for 5 years. They also sought to identify prognostic factors for late death to improve risk-stratified care during follow-up.
The researchers used the SEER database to identify 1,351 patients diagnosed with Ewing’s sarcoma between 1973 and 2013.
Median follow-up for these patients was 11.75 years (range, 5-39.75 years).
Overall, 209 patients died, 69% of whom died of Ewing’s sarcoma. Of 5-year Ewing’s sarcoma survivors, 87.5% (95% CI, 85.4-89.3) achieved 10-year OS.
The cumulative incidence of death of Ewing’s sarcoma was 9.9% (95% CI, 8.3-11.8) at 10 years, 14.1% (95% CI, 11.9-16.8) at 20 years and 16% (95% CI, 13.2-19.2) at 30 years.
Significant univariate adverse prognostic factors included age of 18 years or older at diagnosis, male sex, or having the primary tumor site in the axial and pelvis or pelvic region.
A smaller proportion of those diagnosed as an adult (aged 18 years or older) achieved 10-year OS 82% (95% CI, 78.9-85.9) than those diagnosed when aged 17 years or younger (90.7%; 95% CI, 88.2-92.7).
The OS disparity between adult age vs. adolescent age at diagnoses persisted at 20 years (70.2% vs. 85.2%) and 30 years (62.3% vs. 77.8%).
Of the male population, 85.9% (95% CI, 83-88.4) achieved 10-year OS, 76.2% (95% CI, 71.6-80.2) achieved 20-year OS and 69.4% (95% CI, 62.6-75.2) achieved 30-year OS. A greater proportion of females survived at all these time points (10 years, 89.5%; 95% CI, 86.4-91.9; 20 years, 83%; 95% CI, 78.4-86.8; 30 years, 74.5%; 95% CI, 66.9-80.6).
A smaller proportion of patients with primary tumors in the axial vs. nonaxial/nonpelvic tumors and pelvis achieved 10-year OS (85.1% vs. 90.8%), 20-year OS (77.4% vs. 81.8%) and 30-year OS 66.4% vs. 77%).
Further, a smaller proportion of patients with primary tumors specifically in the pelvic region vs. nonpelvic region achieved 10-year OS (82.4% vs. 88.7%), 20-year OS (74.4% vs. 80.3%) and 30-year OS (65.5% vs. 72.8%)
Upon multivariable analysis, independent adverse prognostic factors for late death in this population included black race (HR = 2.16; 95% CI, 1.2-3.88), age of 18 years or older at diagnosis (HR = 2.02; 95% CI, 1.53-2.67), male sex (HR = 1.43; 95% CI, 1.08-1.91) and primary tumor site in the axial and pelvis (HR = 1.43; 95% CI, 1.07-1.91).
Limitations of this study included an inability to include tumor size — which has been identified as prognostic in previous studies — because 44% of patients in this population were missing these data. Additionally, patients with disease relapse who were alive at 5 years were not identified as such and were analyzed without distinction.
Finally, SEER data are limited regarding treatment modalities in the initial management of the patients, so the analysis focused exclusively on prognostic factors at baseline and not treatment-related factors that could be associated with late death.
“Understanding the biology of these late recurrent tumors will be important to improve our insight into mechanisms of latency that enable low-level occult disease to persist for many years,” DuBois and colleagues wrote.
Further, a propensity toward relapse, coupled with findings from other studies that indicate a genetic predisposition to developing Ewing’s sarcoma, indicates that late events could potentially stem from a second primary tumor, according to the researchers. – by Anthony SanFilippo
Disclosure: HemOnc Today was unable to confirm the researchers’ relevant financial disclosures at the time of reporting.
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