Abstract
Primary leiomyosarcoma of kidney is rare with aggressive behavior. A 61 year female presented with complaint of right flank pain since 2 months. Radiological investigation show mass in mid and lower pole of right kidney for which she underwent radical nephrectomy. Microscopy reveal malignant mesenchymal tumor and IHC confirmed the diagnosis of renal leiomyosarcoma with Caldesmon, smooth muscle actin [SMA] positive and Ki67% - 25%. Complete resection done and on follow up patient is doing well.
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