Leiomyosarcoma of the inferior vena cava. Diagnosis and surgical management

Leiomyosarcoma of vascular origin are rare tumors arising most frequently from the inferior vena cava (IVC). We report on three patients one of whom underwent definitive resection. These tumors most commonly involve the upper segment of the IVC, and appear with manifestations of the Budd-Chiari syndrome. Lesions at this level are not amenable to surgical therapy. Tumors of the middle and lower segments of the IVC usually cause right-sided pain. Diagnosis is difficult, but is best approached preoperatively by angiography and vena cavography. Optimal therapy of lesions at these levels is surgical resection. Resection of the IVC below the hepatic veins is possible with renal function preserved by collateral drainage of the left renal vein.