| Summary: | Genitourinary tract liposarcoma is considered as the second most commonly reported type of sarcomas. Renal liposarcoma with tumor invasion to inferior vena cava (IVC) is distinctly rare. This tumor has a relatively indolent clinical course with risk of local recurrences (20%–85% rate) after surgery. Angiomyolipomas (AML) are the most important differential diagnosis because both are large fat-containing lesions. Herein, a patient with upper pole kidney liposarcoma with tumor invasion to renal vein and IVC is presented. The optimal management is radical nephrectomy and IVC tumor resection. Chemotherapy and radiotherapy have not shown significant survival benefit. Keywords: Liposarcoma, Radical nephrectomy, Renal sarcoma
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