Leiomyosarcoma of the inferior vena cava: report of a case and review of the literature.

Leiomyosarcoma of the inferior vena cava is a rare and usually fatal disease. Only 45 cases have been reported in the world literature. The authors add one case and review all previous reports. The disease is one primarily of elderly women, arising from the middle third of the inferior vena cava with a wide range of symptomatology, varying form the Budd-Chiari syndrome to a few asymptomatic cases. The tumor may grow very slowly or occasionally very rapidly, shows extensive local invasion, and metastasizes more frequently than previously believed. While radiotherapy and chemotherapy have not been adequately studied, aggresive surgical intervention using the recent advances in vascular surgery appears to offer long term palliation and hopefully cures of this disease in the future.