Malignant fibrous histiocytoma of the kidney is an extremely rare tumor that isthought to arise from the renal capsule. Radical nephrectomy is the standard treatment for localized disease but 25% of patients die of metastatic disease despite radical surgical resection. In the absence of clear pathogenetic information, adjuvant treatment remains empiric. Herein, we describe the case of a previously healthy 58-year-old woman who underwent radical nephrectomy because of 8-cm solid lesion of the right kidney; pathology revealed a pleomorphic malignant fibrous histiocytoma invading the perinephric fat, stage pT3aN0. She received adjuvant radiotherapy. To date, five years after surgery, she is alive and there is no evidence of disease. To our knowledge, this is the first report of favorable long-term results of surgery and adjuvant radiotherapy in a locally-advanced malignant fibrous histiocytoma of the kidney.

Keywords: Adjuvant radiotherapy, histiocytoma, kidney, long-term, radical surgery, rare tumor.
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