Approaches to Inferior Vena Cava Reconstruction in the Setting of Leiomyosarcoma

IntroductionPrimary vascular leiomyosarcomas (PVLs) are rare, aggressive tumors arising from the smooth muscle cells of large veins, most commonly the inferior vena cava (IVC). Fewer than 500 cases have been reported worldwide, and outcomes remain poor without complete surgical resection. Treatment often requires complex venous and multivisceral reconstruction, but data on operative strategies and outcomes remain limited.MethodsWe reviewed four cases of IVC PVL resected at our institution between 2020 and 2023. Each case involved a distinct anatomic segment of the IVC, requiring individualized approaches to resection and vascular reconstruction.ResultsPatient 1 underwent infrarenal IVC resection with en bloc removal of bilateral renal veins and right renal artery, followed by complex reconstruction using cryopreserved cadaveric vein grafts to preserve bilateral renal function. Patients 2 and 3 presented with pararenal and suprarenal IVC tumors, respectively, and underwent en bloc resection with patch venoplasty using bovine pericardium, avoiding luminal narrowing and preserving caval flow. Patient 4 had a large pararenal IVC tumor with suprarenal atriocaval extension requiring cardiopulmonary bypass and patch venoplasty. She subsequently developed two recurrences, the second requiring thoracoabdominal exposure and interposition IVC bypass with a biologic tube graft. All patients recovered well postoperatively, with patent reconstructions and preserved organ function on surveillance imaging. At follow-up ranging from 12 months to 3 years, three patients remained disease-free, while one experienced recurrence managed with repeat resection.ConclusionsIVC leiomyosarcomas present unique surgical challenges requiring patient-specific operative strategies. Complete resection with negative margins remains the cornerstone of treatment, with vascular reconstruction essential to preserve renal function and venous return. This series contributes to the limited literature by demonstrating the feasibility of both standard (patch venoplasty) and complex (renal vein preservation, interposition grafting) reconstructions, highlighting the importance of individualized, multidisciplinary operative planning to optimize outcomes.