Abstract
Renal angiomyolipoma (AML) is a benign mesenchymal tumor usually confined to the kidney, but rare cases exhibit aggressive behavior with extension into the inferior vena cava (IVC) and right atrium (RA), resulting in significant clinical challenges due to risks such as heart failure and pulmonary embolism. We report a case of a 42-year-old asymptomatic woman with right renal AML demonstrating progressive vascular invasion from the kidney to the IVC and RA over two years. The computed tomography (CT) scan revealed a fat-containing tumor with IVC and RA extension. Surgical management comprised right nephrectomy combined with tumor thrombectomy under cardiopulmonary bypass. The patient underwent successful radical resection without perioperative complications. Histopathological analysis confirmed AML, and postoperative follow-up showed no recurrence or residual tumor, with preserved renal function. This case underscores the rare but critical presentation of renal AML with vascular and cardiac extension, emphasizing the importance of comprehensive imaging for diagnosis and surgical planning. Despite its complexity, radical surgery offers favorable outcomes and is the preferred treatment to prevent life-threatening complications. Vigilant monitoring remains essential for timely intervention in similar cases.