Leiomyosarcoma of the heart is an uncommon primary malignant tumor with poor postoperative survival that may be measured in months. A leiomyosarcoma of the left atrium was diagnosed in a 47-year-old man. Initial admission was for acute pulmonary edema requiring emergency surgery. The tumor involved the left atrial cavity, and a radical resection was performed. Six months later an isolated myxomatous recurrence was detected. Heart transplantation was then performed. The patient is in good health 20 months after operation with no evidence of residual disease or recurrence. The literature has been reviewed. Surgical resection is not an adequate treatment for leiomyosarcoma of the left atrium and early heart transplantation probably offers the only hope for these patients.
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