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Cardiac function in pediatric Ewing sarcoma during and after treatment: A longitudinal study
Radnyi Mande1, Maya Prasad1,2, Shyam Srinivasan1,2
1Paediatric Oncology, Tata Memorial Hospital, Mumbai, India.
Insights
Pediatric Ewing sarcoma patients treated with anthracyclines face a high risk of cardiac dysfunction, with risk factors including older age, female sex, and undernutrition. Lifelong surveillance is crucial for early detection and management of heart issues.
Area of Science:
- Pediatric Oncology
- Cardiology
- Clinical Research
Background:
- Anthracycline therapy improves Ewing sarcoma outcomes but poses risks of severe cardiac dysfunction.
- Evaluating the prevalence and contributing factors of cardiac dysfunction in pediatric Ewing sarcoma (pES) is critical.
Purpose of the Study:
- To assess the incidence and identify determinants of cardiac dysfunction in pediatric patients with Ewing sarcoma.
Main Methods:
- Retrospective analysis of 650 pediatric patients (0-18 years) treated with the EFT 2001 protocol (anthracycline-containing regimen).
- Cardiac dysfunction defined as left ventricular ejection fraction <50%.
- Data collected from January 2001 to December 2018.
Main Results:
- 13% of patients (85/650) developed cardiac dysfunction at a median of 13 months post-treatment.
- Cumulative incidence reached 15% by 10 years; 24.7% of affected patients experienced LV function normalization.
- Risk factors identified: older age at diagnosis, female sex, undernutrition, and chest wall tumor location.
Conclusions:
- Pediatric Ewing sarcoma patients exhibit a significant incidence of cardiac dysfunction, persisting long after therapy.
- Undernutrition is a key risk factor, necessitating close monitoring in affected children.
- Long-term cardiac surveillance is essential for managing potential long-term complications.
Background:
While anthracycline therapy has been shown to improve outcomes in Ewing sarcoma, it may be associated with severe and even fatal cardiac dysfunction. We evaluated the burden and determinants of cardiac dysfunction in pediatric Ewing sarcoma (pES).
Methods:
This retrospective study included children aged 0-18 years with pES treated at our center with the EFT 2001 protocol (anthracycline and cyclophosphamide containing regimen), with/without radiation therapy from January 2001 to December 2018. Cardiac dysfunction was defined as left ventricular (LV) ejection fraction with an absolute value <50%.
Results:
Amongst 650 eligible patients (median age at diagnosis 12 years and median follow-up duration 69 months), 85 (13%) developed cardiac dysfunction, at a median 13 months (range: 1-168 months). The cumulative incidence of cardiac dysfunction was 5.7% at 12 months, 12% at 2 years, 13% at 3 years, 14% at 5 years, and 15 % at 10 years. At a median follow-up duration of 25 (range: 3-212) months, 21 (24.7%) patients had normalization of LV function, whereas nine (10.6%) patients died of cardiac causes. Older age at diagnosis (7-12 years OR 5.1, p = .01, 13-18 years, OR 3.9, p = .03), female sex (OR 2.3, p = .004), undernutrition (OR 2.9, p = .001), and chest wall location (OR 8.7, p = .08) were risk factors for cardiac dysfunction.
Conclusions:
Children with Ewing sarcoma have a high incidence of cardiac dysfunction, which continues to develop even years after therapy, underlining the need for life-long surveillance. Undernourished children are at a higher risk for cardiac dysfunction and need stringent monitoring.
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