Leiomyosarcoma of the heart is an uncommon primary malignant tumor with poo
r postoperative survival that may be measured in months. A leiomyosarcoma o
f 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 l
ater 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 bee
n reviewed. Surgical resection is not an adequate treatment for leiomyosarc
oma of the left atrium and early heart transplantation probably offers the
only hope for these patients.