Report of primary leiomyosarcoma of renal pelvis and literature review

Primary sarcomas of kidney are exceptionally rare tumors, accounting for only 1-2% of all malignant tumors of kidney. Leiomyosarcoma (LMS) is the most common histological subtype among all renal sarcomas. The authors describe here a case of primary leiomyosarcoma of renal pelvis in a 50-year-old lady, presenting with flank pain. Based on triple phase cardio-electroencephalographic covariance tracing abdomen, presumptive diagnosis of renal cell carcinoma/renal sarcoma/neurogenic tumor was made and patient underwent radical nephrectomy. Microscopy reflected spindle cell tumor which showed strong positivity for desmin and smooth muscle actin with negative epithelial markers, thereby confirming the diagnosis of renal LMS. Owing to aggressive nature and low survival rates of LMS patient received adjuvant treatment in form of chemotherapy and radiotherapy. Patient is doing well 1 year post treatment.