RESUMEN
Perivascular epithelioid cell neoplasm (PEComa) is one of the rare entities which is challenging to diagnose clinically. These tumors occur due to tuberous sclerosis complex gene mutations leading to upregulation and overexpression of the mammalian target of rapamycin (mTOR). Malignant PEComas are rare, and we report a peculiar case of PEComa treated with mTOR inhibitors. A 43-year-old woman presented with complaints of back pain, intermittent fever, dysuria, and cough with expectoration for one month. Abdominal computed tomography (CT) revealed heterogeneously enhancing exophytic mass of the left kidney. A positron emission tomography CT whole body showed a primary malignancy in the left kidney, sclerotic lesions in the bony skeleton, and lymphangitis carcinomatosis in both lungs. A biopsy of the left renal mass revealed PEComa, focally positive for melanocytic and muscle markers. She was commenced on treatment with intravenous temsirolimus, and there was a complete tumor regression by the end of the completion of six cycles.