ABSTRACT
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 gloomy prognosis. We describe a 40 year-old white female complaining of abdominal fullness for 7 weeks before she sought medical assistance. Initial work-up including sonography and computed tomography showed a huge tumoral mass in the abdominal cavity seemingly originating from the IVC with displacement of the right ureter and hydronephrosis. The patient underwent surgical resection of the tumor. Pathologic diagnosis was leiomyosarcoma. Postoperatively, she was placed on coumadin and adjuvant chemotherapy was started. Considering the aggressiveness of this tumor, early radical enblock resection with clear margins is still the only chance for long-term survival