An insight into the inferior vena cava leiomyosarcoma

Abstract

Primary vascular leiomyosarcoma (LMS) is a rare tumor accounting for 2% of all LMSs, with the most common site being inferior vena cava (IVC). Overall prognosis is poor, with a median survival of 2 years only. Definitive treatment strategy has not been defined yet, due to the availability of small case series or reports only. However, extrapolating from the treatment of other sarcomas, definitive surgery followed by adjuvant treatment in the form of radiation and chemotherapy is usually followed. This review article focuses on presentation, diagnostic workup, the treatment options for IVC LMS, and their effect on the outcome, so that optimal management can be planned for individual cases. For this, a literature PubMed/Medline search was performed from January 1995 to December 2013 and reviewed to define the rare presentation of these vascular tumors, diagnostic workup, surgical options and reconstruction methods available, and the indications for the use of adjuvant chemotherapy and radiation.