Cardiac rhabdomyosarcomas (RMS) are rare malignant tumors of the heart with a poor prognosis, even when appropriate treatment regimens are initiated. We present the case of a 12-year-old male who underwent radical resection of a right atrial mass, which was confirmed to be a primary cardiac RMS. He has received regular follow-up for the last 35 years, representing what is to our knowledge the longest reported disease-free survival in a patient with a primary cardiac RMS.
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