Intravenous leiomyomatosis with intracardiac extension

Intravenous leiomyomatosis is a benign smooth muscle tumor of uterine origin characterized by grossly visible masses of smooth muscle cells growing within venous channels. A case of leiomyomatosis arising in the uterine veins with extension into the right iliac veins, vena cava, right atrium, right ventricle and into the pulmonary outflow tract is described. The tumor was completely and successfully removed by a staged approach with median sternotomy followed by laparotomy several days later. A brief review of the literature and a discussion of the operative approach are presented.