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

Q: What is the clinical evidence from this study?

Study design: Case Report. Population: Primary extraskeletal cardiac Ewing sarcoma (n=1). Intervention: Multimodal therapy (surgical resection, chemotherapy, radiotherapy). Primary outcome: Survival duration.

Q: What does this research mean for the field?

Aggressive multimodal therapy resulted in prolonged survival for a patient with primary cardiac Ewing sarcoma, emphasizing the need for timely diagnosis in young patients. Novelty: ClaimNovelty.NOVEL_FINDING. Consensus alignment: ConsensusAlignment.NEUTRAL.

Ahmad Khajeh-Mehrizi; Mehdi Karimi; Masoud Mortezazadeh; Bahar Amiri; Hamidreza Zarei; Hana Saffar

doi:10.1186/s13019-025-03774-3

December 26, 2025Journal of Cardiothoracic SurgeryOpen Access

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

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Key result

Multimodal therapy achieves 36-month survival in primary cardiac Ewing sarcoma.

n=1

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Key Points

To report the clinical presentation, diagnosis, and long-term multimodal management of an exceptionally rare primary extraskeletal cardiac Ewing sarcoma.
Evaluated a 27-year-old male presenting with cardiopulmonary and systemic symptoms via CT, MRI, PET-CT, and histopathological analysis.
Confirmed the diagnosis using an immunohistochemical panel evaluating CD99, NKX2.2, and FLI-1 expression.
Administered a multimodal therapeutic regimen comprising surgical tumor resection, protocol-based chemotherapy (VAC/IE), and palliative radiotherapy.
Immunohistochemical profiling confirmed primary extraskeletal Ewing sarcoma of the right ventricular outflow tract, showing strong membranous CD99, NKX2.2, and FLI-1 positivity.
Post-chemotherapy PET-CT documented complete metabolic resolution of the aortopulmonary mass and absence of pericardial effusion.
The patient achieved a survival duration of 36 months following diagnosis, exceeding the historical median survival of approximately 12 months reported for primary cardiac Ewing sarcoma.

Study Design

Type

Case Report (n=1)

Multicenter

No

Structured PICO

P

Population

A 27-year-old male with primary extraskeletal cardiac Ewing sarcoma treated with multimodal therapy and followed for 36 months.

I

Intervention

Multimodal therapy including surgical resection, RVOT reconstruction, pulmonary valve replacement, chemotherapy (VAC/IE: vincristine, actinomycin-D, cyclophosphamide, ifosfamide, etoposide), and radiotherapy

O

Outcome

Survival durationhard clinical

Aggressive multimodal therapy including surgery, chemotherapy, and radiotherapy can lead to prolonged survival in rare cases of primary cardiac Ewing sarcoma.

Limitations

Molecular confirmation of Ewing sarcoma (EWSR1 rearrangements) was not performed due to financial constraints.
Molecular confirmation of Ewing sarcoma was not performed due to financial constraints

Implication

May support multimodal therapy consideration in primary cardiac Ewing sarcoma; hypothesis-generating from single case and leaves open need for validation.

Abstract

BACKGROUND: Ewing sarcoma (ES) is a malignant small round-cell tumor that typically arises in the bones or soft tissue of children and young adults. Although most cases involve the skeletal system, extraskeletal presentations are less common, especially involving visceral organs. Primary cardiac ES is exceptionally rare, with only a limited number of cases reported in the literature. CASE PRESENTATION: A 27-year-old male presented with symptoms including cough, dyspnea, fever, anorexia, night sweats, and weight loss. Clinical and imaging evaluations revealed a mass anterior to the right ventricular outflow tract (RVOT), which was ultimately diagnosed as primary extraskeletal cardiac ES. The patient was treated with a comprehensive multimodal approach, incorporating surgical resection, chemotherapy, and radiotherapy. Remarkably, he survived for 36 months after diagnosis, surpassing the survival durations reported in most previous cases. CONCLUSION AND KEY CLINICAL MESSAGE: 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.

Discussion

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Cite This Study

Khajeh-Mehrizi et al. (2025) conducted a case report in Primary extraskeletal cardiac Ewing sarcoma (n=1). Multimodal therapy (surgical resection, chemotherapy, radiotherapy) was evaluated on Survival duration. Aggressive multimodal therapy including surgical resection, chemotherapy, and radiotherapy achieved a prolonged survival of 36 months in a patient with primary cardiac Ewing sarcoma.

synapsesocial.com/papers/6963221f91e05aa366cb89ef https://doi.org/10.1186/s13019-025-03774-3

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

Figures

Key Points

Study Design

Structured PICO

Limitations

Implication

Abstract

Discussion

Cite This Study

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

Figures

Key Points

Study Design

Structured PICO

Limitations

Implication

Abstract

Discussion

Cite This Study

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