Primary cardiac undifferentiated pleomorphic sarcoma is rare and is associated with very poor survival. We report a case of a 45-year-old female who presented with dyspnea on effort, in whom an echocardiographic exam showed a large mass in the left atrium and the tumor resection was performed. The pathological diagnosis of the resected tumor was undifferentiated pleomorphic sarcoma which subsequently recurred. The patient needed four re-surgeries, and chemotherapy with Pazopanib was performed. A long-term survival of 5 years after the initial surgery was achieved.

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.