This study states that On computed tomography of the abdomen and pelvis, a large retroperitoneal mass compressing and potentially involving the distal inferior vena cava (IVC) was identified. This was better characterized as a complex soft tissue mass measuring 6.8 × 7.3 cm on follow-up magnetic resonance imaging, with suspected origin from the IVC confluence. The patient was taken for curative R0 resection with venous reconstruction of the IVC and iliac vein confluence. The patient tolerated the procedure well, undergoing reconstruction of the IVC and bilateral iliac veins with 16-mm polytetrafluoroethylene. Final pathologic examination showed a primary IVC/iliac vein leiomyosarcoma, grade III, with no violation of the capsule and negative margins. The patient had prolonged ileus postoperatively but was successfully discharged on anticoagulation on postoperative day 11. At 3-month follow-up, his reconstruction is widely patent, and leg swelling and neuropathy have resolved. Radical en bloc resection remains the “gold standard” treatment of retroperitoneal tumors. This case demonstrates the multidisciplinary care of rare soft tissue tumors involving the central veins, notably in this case arising from the IVC confluence. We present a case of oncologic resection of biopsy-proven leiomyosarcoma arising from the IVC confluence.


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