Background: Inferior vena cava (IVC) leiomyosarcoma is a rare tumor of smooth muscle origin. It is often large by the time of diagnosis and may involve adjacent organs. A margin-free resection may be curative, but the resection must involve the tumor en bloc with the affected segment of vena cava and locally involved organs. IVC resection often requires vascular reconstruction, which can be done with prosthetic graft. Case presentation: We describe a 39-year-old man with an IVC leiomyosarcoma that involved the adrenal gland, distal pancreas, and blood supply to the spleen and left kidney. Tumor excision involved en bloc resection of all involved organs with reimplantation of the right renal vein and reconstruction of the IVC with a polytetrafluoroethylene graft. The patient recovered without renal insufficiency, graft infection, or other complications. Follow-up abdominal imaging at 1 year showed a patent IVC graft and no locally recurrent tumor. Prosthetic graft provides a sufficient diameter and length for replacement conduit in extensive resection of IVC leiomyosarcoma. Conclusion: To our knowledge, this is the first case of resection of an IVC sarcoma with prosthetic graft reconstruction in combination with pancreatic resection. Aggressive surgical resection including vascular reconstruction is warranted for select IVC tumors to achieve a potentially curative outcome.
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