Primary synovial sarcoma of the heart is a rare tumor, with only six previous cases having been reported in the literature. Treatment has included surgery with or without chemotherapy. We present the first case of a documented synovial sarcoma arising from the pericardium in a 19-year-old man. Molecular analysis for t(X; 18) SYT-SSX gene fusion was positive. Radiation treatment was given postoperatively to the entire heart with a boost to the area where the margins were positive.
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