A 63-year-old diabetic and hypertensive lady presented in New York Heart Association class III–IV dyspnea on exertion. Echocardiography showed a large mass attached to the anterior mitral leaflet and the base of the interatrial septum. After removal of the mass and excision of the anterior and posterior mitral leaflets, a bioprosthetic valve was deployed. The postoperative course was uneventful. Histopathology showed that the tumor was a high-grade rhabdomyosarcoma. Although it is a highly lethal tumor, surgical removal was indicated to relieve dyspnea, clarify the diagnosis, and improve short-term survival. Our patient survived for 8 months after surgical excision.
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