Abstract
Approximately 10% of soft tissue sarcomas are synovial sarcomas, and 90% of these occur in the extremities. Among the primary tumors in the heart, 25% are malignant. Primary synovial sarcoma of the heart is an extremely rare entity. A myriad of investigations such as histopathology, immunohistochemistry, electron microscopy, and molecular genetic techniques are required for confirmation of the diagnosis. The tumor is nearly always lethal, but surgical resection with chemotherapy may prolong the life of the patient. We describe the case of a young patient with a primary synovial sarcoma arising from the right ventricle.
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