| Summary: | A synovial sarcoma (also known as malignant synovioma) is a rare form of cancer that usually occurs near the joints of the arm, neck, or leg. It is one of the soft tissue sarcomas. Primary cardiac neoplasms are rare. Most common site for synovial sarcoma is lower limb. Synovial sarcoma of the heart is extremely rare. Occurrence of synovial sarcoma at extra synovial site is very uncommon. It is two times common in right side heart than left side so left side synovial sarcoma is rarest of rare, only very limited cases are reported. Here, we report a case of primary synovial cell sarcoma of the left side of the heart in a 26-year-old female. She presented with dyspnea and weakness only. She was operated for left-sided mass covering mitral valve; further histo-pathology revealed the mass as synovial cell sarcoma. In Indian scenario, this type of rare case needs documentation.
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