Leiomyosarcoma of level II inferior vena cava—an original solution for bilateral renal vein reconstruction

The leiomyosarcoma of the inferior vena cava (IVC) is a rare clinical entity, although it represents the most common malignant tumor of the venous system. Level II IVC tumors (supra-renal) are the most frequent and those who have a better prognosis for the development of symptoms earlier.Case reportThe authors report a case of IVC leiomyosarcoma in a 59-year-old woman, presenting with DVT of the right lower limb, subsequent to prolongued nonspecific abdominal pain. Computed tomography revealed a large retroperitoneal neoformation, centered to IVC, which extended above the renal veins (the left one patent and the right one involved in the mass). The patient underwent en block resection of the tumor and reconstruction of the renal veins: construction of a new IVC bifurcation at the supra-renal level with a bifurcated PTFE graft, followed by graft extension to both renal veins using externally-supported 8mm PTFE grafts. Histology revealed a high-grade leiomyosarcoma. The postoperative period was complicated by a type 2 MI and retroperitoneal hematoma, with occlusion of the right graft branch and partial infarction of the right kidney. The patient underwent surgery again and proceeded to partial resection of the thrombosed graft branch. The patient was discharged home under anticoagulation and is clinically well without edema of the lower limbs, normal renal function, and has begun adjunctive therapy.ConclusionThe prognosis of these tumors is poor, with a high recurrence rate. An aggressive surgical approach combined with adjuvant therapy may not be curative, but is the best strategy to prolong survival