What question did this study set out to answer?

To present a rare case of primary cardiac Ewing sarcoma and its prolonged survival outcome.

January 10, 2026Journal of Cardiothoracic Surgery0 citationsOpen Access

Primary cardiac ewing sarcoma presenting as an infiltrative myocardial mass: a rare case with long-term survival

AKAhmad Khajeh-MehriziImam Khomeini Hospital MKMehdi Karimi MMMasoud MortezazadehImam Khomeini Hospital

Key Result

Aggressive multimodal therapy resulted in prolonged survival for a patient with primary cardiac Ewing sarcoma, emphasizing the need for timely diagnosis in young patients.

Key Points

To present a rare case of primary cardiac Ewing sarcoma and its prolonged survival outcome.
Aggressive multimodal therapy was utilized for treatment.
A thorough differential diagnosis was performed for intracardiac tumors.
Focus on younger patients presenting with systemic symptoms.
Prolonged survival was achieved following treatment.
Ewing sarcoma was successfully identified as the cause of myocardial mass.
Case highlights the importance of early intervention.

Structured PICO

Population

1 adult (27-year-old male) with primary extraskeletal cardiac Ewing sarcoma presenting with a mass anterior to the right ventricular outflow tract (RVOT) and reduced ejection fraction (35-40%).

Intervention

Surgical resection, RVOT reconstruction, and pulmonary valve replacement, followed by VAC/IE chemotherapy (Vincristine, Actinomycin-D, Cyclophosphamide alternating with Ifosfamide and Etoposide) and targeted radiotherapy.

Outcome

Survival and tumor remissionhard clinical

Aggressive multimodal therapy including surgery, chemotherapy, and radiotherapy can achieve prolonged survival and remission in patients with rare primary cardiac Ewing sarcoma.

Main Result

Absolute Event Rate: 0% vs 0%

Abstract

A prolonged survival outcome was demonstrated in this rare case of cardiac ES through aggressive multimodal therapy. Although rare, ES should be included in the differential diagnosis of intracardiac tumors, especially in younger patients presenting with systemic symptoms, to facilitate timely diagnosis and intervention.

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