Abstract
Primary leimyosarcoma of the kidney is extremely rare. The management is unclear due to the limited literature on the subject. The diagnosis should be considered when imaging shows a relatively hypovascular tumor involving one renal pole, with atypical features on microscopy. We report a renal leiomyosarcoma in a 68 year old man, who underwent right radical nephrectomy. A tumor-free surgical margin is the most important prognostic factor. Although various modalities of adjuvant treatment have been tried, the overall prognosis is poor as the tumor is highly aggressive.
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