Primary cardiac malignant tumor is rare and is associated with very poor survival. We report a case of a 45-year-old female who presented with dyspnea and general edema due to severe congestive heart failure, in whom an echocardiographic exam showed a large mass in the left atrium, mitral valve regurgitation and tricuspid valve regurgitation and the tumor resection, mitral valve repair and tricuspid annuloplasty were performed under semi-emergency. The pathological diagnosis of the resected tumor was cardiac intimal sarcoma which recurred; the patient needed re-surgery after 1 year and 5 months, and chemotherapy by pazopanib was performed. She died due to widespread metastasis. A relatively long-term survival of two years and 1 month after the initial surgery was achieved.
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