Leiomyosarcoma of the left kidney with renal vein and inferior vena cava tumor thrombus

Primary renal leiomyosarcoma (PRLMS) are rare, aggressive smooth muscle tumors, and vascular involvement with tumor thrombus is especially uncommon. We present a case of a 66-year-old male with hematuria and abdominal pain found to have a 10.5 cm left renal mass with thrombus extending into the renal vein and inferior vena cava (IVC). The patient underwent open radical nephrectomy with IVC thrombectomy. Histopathology confirmed grade 2 leiomyosarcoma with negative surgical margins, The patient recovered uneventfully and remains disease-free five months postoperatively, highlighting the important of complete resection.