Recurrent intravenous leiomyosarcoma of the uterus in the retrohepatic vena cava

Although intravenous extension of uterine leiomyosarcomas has been described, extension into the inferior vena cava (IVC) and right atrium, so-called ‘intravenous leiomyosarcomatosis (IVLS)’, is rare. To our knowledge only a few cases have been described in the literature. We describe a case of recurrent uterine leiomyosarcoma to the retrohepatic IVC. The patient was initially treated with total abdominal hysterectomy. Follow-up computed tomography a year later showed an extensive intravascular and intracardiac soft tissue mass treated with tumor extraction using cardiac bypass. Five years later she presented to our institution with a new retrohepatic caval mass treated with surgical resection and caval grafting. IVLS is a rare disease that is best treated with surgical resection even in the recurrent setting. The role of adjuvant therapy remains unclear.