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What Do We Know about Survival in Skeletally Premature Children Aged 0 to 10 Years with Ewing Sarcoma? A Multicenter
Sarah E Bosma1, Lizz van der Heijden1, Luis Sierrasesúmaga2
1Department of Orthopedic Surgery, Leiden University Medical Center, 2333 ZA Leiden, The Netherlands.
Insights
Young children with Ewing sarcoma (ES) have excellent survival rates, with wide resection margins significantly improving outcomes. This study highlights the effectiveness of current treatments for pediatric ES patients.
Area of Science:
- Pediatric Oncology
- Sarcoma Research
- Clinical Outcomes
Background:
- Younger age is linked to better overall survival (OS) in Ewing sarcoma (ES).
- Minimizing treatment burden and late sequelae is crucial for young patients.
- This study focuses on the clinical characteristics, treatment, and outcomes of ES patients aged 0-10 years.
Purpose of the Study:
- To describe the clinical characteristics, treatment, and outcomes of Ewing sarcoma patients aged 0-10 years.
- To identify factors influencing overall survival in this pediatric cohort.
- To assess local recurrence-free survival (LRFS) and distant metastasis-free survival (DMFS).
Main Methods:
- Retrospective multicenter study including 60 patients aged 0-10 years.
- Data collected from sarcoma centers in the Netherlands and Spain (1982-2008).
- Minimum follow-up of 10 years; analysis of OS, LRFS, and DMFS, and influencing factors.
Main Results:
- 10-year OS, LRFS, and DMFS were 81%, 89%, and 81%, respectively.
- Limb salvage was achieved in 93% of patients.
- A wide resection margin was significantly associated with better OS; younger age (<6 years) and smaller tumor volume (<200 mL) showed trends towards better survival.
Conclusions:
- Youngest Ewing sarcoma patients (0-10 years) demonstrate very good overall survival.
- Limb salvage surgery is highly achievable (>90%) in this age group.
- Wide resection margin is the sole significant factor associated with improved survival in pediatric ES.
Abstract:
(1) Background: Younger age has been associated with better overall survival (OS) in Ewing sarcoma (ES), especially under the age of 10. The favorable survival in younger patients underlines the need for minimizing treatment burden and late sequelae. Our study aimed at describing clinical characteristics, treatment and outcome of a cohort of ES patients aged 0−10. (2) Methods: In this retrospective multicenter study, all consecutive ES patients aged 0−10, treated in four sarcoma centers in the Netherlands (n = 33) and one in Spain (n = 27) between 1982 and 2008, with a minimum follow-up of 10 years, were included. OS, local recurrence-free survival (LRFS) and distant metastasis-free survival (DMFS) were calculated. Potential factors of influence on OS (risk and protective factors) were analyzed. (3) Results: 60 patients with median follow-up 13.03 years were included. All patients were treated with chemotherapy in combination with local treatment, being surgery alone in 30 (50%) patients, radiotherapy (RT) alone in 12 (20%) patients or surgery plus RT in 18 (30%) patients (12 pre- and 6 postoperative). Limb salvage was achieved in 93% of patients. The 10-OS, -LRFS and -DMFS are 81% (95% CI: 71−91%), 89% (95% CI: 85−93%) and 81% (95% CI: 71−91%), respectively. Six patients developed LR, of which two developed subsequent DM; all had axial ES (pelvis, spine or chest wall), and these patients all died. Ten patients developed DM; eight died due to progressive disease, and two are currently in remission, both with pulmonary metastasis only. Negative or wide resection margin was significantly associated with better OS. Age < 6 years, tumor volume < 200 mL, absence of metastatic disease and treatment after 2000 showed trends towards better OS. Two patients developed secondary malignancy; both had chemotherapy combined with definitive RT for local treatment. (4) Conclusions: Overall survival of these youngest patients with ES was very good. Limb salvage surgery was achieved in >90% of patients. Wide resection margin was the only factor significantly associated with better survival.
