Primary leiomyosarcoma of the inferior vena cava with Budd-Chiari syndrome

A 44-year-old Japanese woman with leiomyosarcoma of the inferior vena cava is reported. She presented with Budd-Chiari syndrome and died of hepatic failure about 3 months after the onset of symptoms. The tumor arose from the middle segment of the inferior vena cava, occluded the inferior vena cava and projected into the right atrium. A total of 28 cases of Budd-Chiari syndrome due to primary leiomyosarcoma of the inferior vena cava are reviewed.