Primary malignancies of the aorta are extremely rare. A review of the
literature indicates that 35 documented cases of primary tumors of the
aorta have been reported over the past 120 years. The histologic and
morphologic characteristics of these lesions may be variable. In this
case, progressive claudication of the left leg and buttocks with absen
t femoral pulses in a middle-aged woman was found to be a primary leio
myosarcoma of the abdominal aorta. A magnetic resonance imaging study
defined a retroperitoneal space-occupying mass on the left side of the
aorta at the level of the fourth lumbar vertebrae. A magnetic resonan
ce angiographic scan of the abdominal aorta and an aortogram revealed
total occlusion of the distal abdominal aorta with reconstitution at t
he level of the common femoral arteries bilaterally, with normal vesse
ls more distal to that region, The patient underwent surgical explorat
ion and resection of the retroperitoneal, infrarenal, occluding aortic
mass. The mass was found to be a high-grade sarcoma displaying smooth
muscle cell differentiation. The resection of this lesion, perioperat
ive management, and pathologic characteristics of a rare primary neopl
asm of the aorta are discussed in this review.