Abstract
Background: Leiomyosarcoma originating from the renal vein (RVLMS) is extremely rare. Only few cases are reported in the literature. Case Report: We report here an exceptional case of a huge LMS of the right renal vein incidentally discovered, occurring in a 73-year-old female patient. Conclusions: This tumor lacks specific clinical manifestations and specific imaging features. Only pathological features make their diagnosis. Standard treatment consists of radical nephrectomy followed by chemotherapy and/or radiotherapy. The prognosis factors are not well identified. Overall prognosis of renal vein LMS is poor
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