Malignant renal epithelioid angiomyolipoma with an inferior vena cava and right atrium thrombus

The epithelioid variant type of angiomyolipomas presents malignant characteristics compared to classic angiomyolipomas. Epithelioid angiomyolipomas (eAMLomas) are easy to misdiagnose as renal cell carcinoma due to similar histologic and radiographic findings. Until recently, only a few cases of distant metastases of eAMLoma were reported. Herein, we describe a 69-year-old woman with renal eAMLoma and tumor thrombus metastasis to the renal vein, inferior vena cava, and right atrium. She received a left radical nephrectomy combined with tumor thrombus excision and systemic chemotherapy. Using this case, we remind readers of the aggressive behavior and poor prognosis of eAMLomas and review the literature on the optimal treatment strategy.