Primary angiosarcoma of the kidney: a challenging diagnosis

Angiosarcoma is a rare malignancy derived from endothelial cells which behaves aggressively. Primary angiosarcoma of the kidney is even rarer, and its clinical and radiological presentations do not differ from clear cells renal cell carcinoma (ccRCC). Management protocols are not standardized, although nephrectomy is usually performed. Subsequent treatments (chemotherapy, radiotherapy, and lately, targeted therapies) vary considerably. Herein, we report the case of a middle-aged patient harboring primary angiosarcoma of the left kidney and discuss its presentation and management in light of current guidelines. The case is described for its rarity and masquerading nature.