Abstract:

Undifferentiated primary pleomorphic sarcoma of the kidney is a very rare finding. Renal 
sarcoma accounts for 1-3% of all renal carcinomas. The tumor has an aggressive nature, rapid 
progression and metastasizes via lymphatic and hematogenous routes. 
The aim of the report is to present a rare renal tumor and the diagnostic and treatment algorithm in 
the patient. To discuss the demographic picture of renal cell carcinoma worldwide and in Bulgaria, 
as well as the need for screening programs for this nosological entity. 
Radical nephrectomy remains the gold standard in the treatment of the disease. Histological 
analysis is essential for making an accurate diagnosis. Active dispensary surveillance is an 
important component of the postoperative treatment plan, as well as the discussion of including 
adjuvant chemotherapy and radiotherapy. 

Keywords: kidney, sarcoma, rare, aggressive, hemorrhage

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