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"Second" Primary Cardiac Sarcoma in a Patient With Ewing Sarcoma. Always Expect The Unexpected
Antonino Di Franco1, Mario Gaudino1, Jonathan W Weinsaft2
1Department of Cardiothoracic Surgery, Weill Cornell Medicine, New York, New York.
The Annals of Thoracic Surgery
|January 23, 2017
Summary
When an intracardiac mass is found, doctors usually suspect relapsed Ewing sarcoma. However, this case highlights the rare possibility of a second primary sarcoma, specifically low-grade myxoid spindle cell sarcoma, co-occurring with Ewing sarcoma.
Area of Science:
- Oncology
- Pathology
- Cardiovascular Medicine
Background:
- Ewing sarcoma is a rare bone and soft tissue cancer primarily affecting children and young adults.
- Intracardiac masses in patients with a history of Ewing sarcoma typically raise concern for metastatic disease.
- Distinguishing between metastatic disease and a new primary tumor is crucial for appropriate patient management.
Observation:
- A young patient with a recent history of Ewing sarcoma presented with an intracardiac mass.
- Standard diagnostic suspicion focused on metastatic Ewing sarcoma.
- Further investigation revealed the intracardiac mass to be a distinct, primary low-grade myxoid spindle cell sarcoma.
Findings:
- This report details the first documented case of concomitant diagnosis of Ewing sarcoma and a separate primary low-grade myxoid spindle cell sarcoma in the same patient.
- The findings challenge the assumption that intracardiac masses in Ewing sarcoma survivors are always metastatic.
- Histopathological and molecular analyses confirmed the distinct origins of the two sarcomas.
Implications:
- This case underscores the importance of considering rare differential diagnoses, including secondary primary malignancies, in patients with a history of cancer.
- Accurate diagnosis is critical for guiding treatment strategies and improving patient outcomes in complex oncological scenarios.
- Further research into the co-occurrence of distinct primary sarcomas may elucidate underlying genetic or environmental factors.

