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Outcome in 43 children presenting with metastatic Ewing sarcoma: the St. Jude Children's Research Hospital
C Sandoval1, W H Meyer, D M Parham
1Department of Hematology-Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38101-0318, USA.
Insights
Metastatic Ewing sarcoma survival improved significantly after 1979, with about one-third of patients achieving long-term survival. Advances in therapy, particularly doxorubicin and ifosfamide/etoposide combinations, show promise for improving outcomes in this challenging pediatric cancer.
Area of Science:
- Pediatric Oncology
- Sarcoma Research
- Clinical Outcomes Analysis
Background:
- Ewing sarcoma is a rare bone cancer primarily affecting children and young adults.
- Metastatic Ewing sarcoma at diagnosis significantly impacts patient prognosis.
- Understanding factors influencing survival in metastatic cases is crucial for treatment advancement.
Purpose of the Study:
- To review the St. Jude Children's Research Hospital experience with metastatic Ewing sarcoma over 30 years.
- To analyze the impact of primary tumor characteristics and metastatic sites on survival.
- To evaluate the effect of therapeutic advances on survival rates.
Main Methods:
- Retrospective review of 43 patients with metastatic Ewing sarcoma diagnosed between 1973 and 2003.
- Analysis of patient data including primary tumor site/size, metastatic sites, and treatment era.
- Statistical comparison of survival rates based on various clinical and therapeutic factors.
Main Results:
- Overall survival for the 43 patients was 35%.
- Survival rates showed a significant improvement for patients treated after 1979 compared to those treated before (P = 0.0002).
- No significant differences in survival were observed based on primary tumor site, size, or metastatic site.
Conclusions:
- Approximately one-third of patients with metastatic Ewing sarcoma can achieve long-term survival.
- Therapeutic advances, including intensified doxorubicin doses and the ifosfamide/etoposide combination, may benefit patients.
- Clinical trials exploring intensified chemotherapy regimens are warranted for metastatic Ewing sarcoma.
Abstract:
The purpose of this work was to review the St. Jude Children's Research Hospital experience of patients presenting with metastatic Ewing sarcoma over a 30-year period. Forty-three of 212 cases of Ewing sarcoma presented with metastases at diagnosis. These patients were analyzed to determine whether primary tumor site or size, metastatic site(s), or advances in therapy have had a positive impact on survival. The overall survival for our 43 patients was 35% (95% confidence intervals, 20% to 50%). Comparing patients treated prior to 1979 with those treated after 1979, the overall survival was significantly different (P = 0.0002). Comparing overall survival between pelvic and non-pelvic primaries (P = 0.24), among metastatic sites (P = 0.83), and between tumors measuring > 8 cm in diameter to tumors measuring < 8 cm in diameter (P = 0.12), no significant differences were observed. Approximately one-third of patients presenting with metastatic Ewing sarcoma may achieve long-term survival. Children with metastatic Ewing sarcoma may benefit from clinical trials which intensify the doses of doxorubicin, and the highly effective combination of ifosfamide/etoposide.
