Leiomyosarcoma of the infra-renal inferior vena cava

Leiomyosarcoma of the inferior vena cava (IVC) is a rare slow-growing retroperitoneal tumor. Two percent of leiomyosarcomas are vascular in origin, and tumors of the IVC account for the majority of the cases. The diagnosis is frequently delayed, because affected patients remain asymptomatic for a long period. It has an extremely poor prognosis, with 5-year actuarial malignancy-free survival rates of 30% to 50% after a wide surgical resection. The authors present the case of a patient with IVC leiomyosarcoma who underwent en bloc resection of the tumor along with the involved segment of the infrarenal IVC without caval reconstruction. Complete surgical resection offers the only potential of long-term survival, but survival of unresected patients is generally measured in months. Palliative resections may temporarily improve symptoms but do not offer long-term survival.