Leiomyosarcoma of the inferior vena cava is a rare tumor that is usually fatal. The tumor may grow very slowly or occasionally very rapidly, shows extensive local invasion, and metastasizes more frequently than previously believed. Complete surgical resection remains the only potential curative therapeutic option. The aim of this study was to report the clinical experience in the management of a patient with leiomyosarcoma. A 65-year-old woman with a history of vague abdominal pain and leg swelling underwent computed tomography which demonstrated an occlusion of the inferior vena cava. The patient received a complete excision of the tumor without reconstruction and histological analysis confirmed the diagnosis of leiomyosarcoma type 1. At 3 years, the patient is still doing well with minimal leg edema and a contrast-enhanced CT demonstrates no evidence of recurrence locally or in distant sites. Leiomyosarcoma is a rare and aggressive tumor that presents with non-specific symptoms. Computerized tomography with 3-D reconstruction is a useful tool to define the presence and entity of the collateral circulation and therefore to decide on the surgical strategy. Resection probably offers the best opportunity for long-term survival.
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