Ieiomyosarcoma of the superior vena cava producing superior vena cava syndrome and heart tamponade

An autopsy case of primary leiomyosarcoma arising in the superior vena cava is presented. A 44 year old Japanese man presented with superior vena cava syndrome and eventually died due to heart tamponade and acute renal failure. Autopsy revealed that the superior vena cava was occluded with a tumor that had invaded the pericardium and right thoracic cavity. Primary caval venous leiomyosarcoma is a rare but lethal disease and most cases arise from the inferior vena cava. This case represents a very rare case of leiomyosarcoma with the rare clinical findings of superior vena cava syndrome and heart tamponade.