Perivascular epithelioid cell tumors are rare mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. They are mostly benign, however, malignant tumors with aggressive behavior and distant metastasis can also occur. The standard treatment is surgical excision. Here, we report a case of a 25-year-old male with a 4 cm-mass located in the inferior pole of the left kidney, treated by surgical excision
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