RESUMEN
Primary tumors arising from great vessels like the aorta, pulmonary artery or inferior vena cava (IVC) are rare. The latter is the commonest site of its occurrence. It arises from the smooth muscle cells of the vessel wall. Aggressive surgical management should be attempted to excise it whenever possible. We describe a case of primary inferior vena cava tumor involving all three segments of the abdominal inferior vena cava infrarenal, suprarenal and retrohepatic vena cava, along with right kidney, right adrenal as well as right hepatic vein and left renal vein. We resected it completely without reconstruction of the IVC. The patient is doing well seven months after surgery without having any renal insufficiency, hepatic insufficiency or leg edema and having optimum quality of life. To our knowledge, this is the first case of such a long segment IVC leiomyosarcoma treated without IVC reconstruction, and despite its extent and concomitant involvement of the right kidney, right adrenal, right hepatic vein and left renal vein, it had a favorable response combining prolongation of survival and satisfactory quality of life.