Leiomyosarcoma of the inferior vena cava (IVC) is a rare sarcoma, but it is the most common primary malignancy of the IVC. It has an extremely poor prognosis. We describe a 60-year-old white female complaining of abdominal fullness for 7 weeks before she sought medical assistance. Initial work-up including sonography, computed tomography, and magnetic resonance showed a tumor in the right upper quadrant of the abdominal cavity originating from the liver with compression of the IVC and displacement of the right kidney. The patient underwent surgical resection of the tumor with clear margins and reconstruction of the IVC using a Dacron tubular graft. Postoperatively, she was placed on Coumadin® and adjuvant chemotherapy was started. Subsequently, the patient developed metastasis into the liver and peripancreatic nodes during the follow-up period. Considering the aggressiveness of this tumor, early radical en block resection with clear margins is still the only chance for long-term survival.
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