We report two cases of primary inferior vena cava (IVC) leiomyosarcoma. The first patient was a 60-year-old female who presented with abdominal pain. The patient was initially diagnosed with a retroperitoneal sarcoma that may have involved the right renal vessels and the IVC. The Vascular Surgery Service was consulted intra-operatively when it became evident that the IVC was primarily involved. The patient was treated with total en-bloc excision of the infrarenal IVC tumor with concomitant interposition polytetrafluoroethylene (PTFE) graft caval replacement. The second patient was a 58-year-old female who presented with general malaise and anemia. This tumor began in the distal infrarenal IVC and extended cephalad to the suprahepatic IVC, ending approximately 2 cm below the right atrium. Intrapericardial IVC clamping was required for cephalad control in this patient, who was then treated with excision of the tumor and concomitant interposition PTFE graft caval replacement with reimplantation of the right renal vein. A review of this rare tumor is presented.
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