Primary renal leiomyosarcoma: Case report and literature review

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.