Primary vascular leiomyosarcoma is a rare tumor, which arises mainly from the inferior vena cava. Clinical signs are non-specific. Systemic metastasis occurs in the late stage. Imaging with color Doppler ultrasonography, contrast-enhanced computed tomography, or magnetic resonance imaging can significantly contribute to the diagnosis. We present a case of leiomyosarcoma of the inferior vena cava that extended into the right renal vein and the right iliac vein, as it is a rare case and emphasize the significance of the imaging methods in its diagnosis.
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