Leiomyosarcoma of the inferior vena cava. Case report and review of the literature.

Leiomyosarcoma of the inferior vena cava is reported with increasing frequency but remains a rare lesion. In this article we report one case and review 69 cases. The disease affects mainly women approximately 50 years of age. The symptoms vary according to the location of the tumor; Budd-Chiari syndrome and leg edema occur in the more centrally located tumors and abdominal pain and swelling are seen in the more peripherally located ones. In 40% of the cases distant metastases are evident at the time of the patients' first presentation and are found most commonly in the liver and/or lungs. After resection the majority of the patients eventually die of metastatic disease with a mean survival of approximately 40 months.