Sarcomas are very rare tumors and in 10-30% of the cases are located in the retroperitoneum. Leiomyosarcomas of inferior vena cava accounts for less than 1% of all sarcomas. Despite the poor prognosis as a whole, some cases with R0 resection might have excellent outcome. So, all attempts for R0 resection should be undertaken. Herein we present a case with tumor located at the border of lower and middle third of the IVC managed by excision, primary suture and preservation of both renal veins. A short review of the basic surgical options and risk factors is given.
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