Primary leiomyosarcoma of the abdominal aorta.

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 absent femoral pulses in a middle-aged woman was found to be a primary leiomyosarcoma 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 resonance angiographic scan of the abdominal aorta and an aortogram revealed total occlusion of the distal abdominal aorta with reconstitution at the level of the common femoral arteries bilaterally, with normal vessels more distal to that region. The patient underwent surgical exploration 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, perioperative management, and pathologic characteristics of a rare primary neoplasm of the aorta are discussed in this review.