A larger tumor size, younger patient's age and higher BMI value correlated with a higher risk of tumor hemorrhage. For tumor sizes less than 7.35 cm, we recommend active surveillance or TAE for hemorrhage prevention. We also suggest that surgical management should be considered for patients with tumors larger than 7.35 cm, symptomatic and progressive AML, or suspicious EAML.

EAML of the kidney is a specific type of renal AML with malignant potentials, where around 22% of the patients present with invasion or metastasis. Higher mitotic activities indicate a greater metastatic potential, with radical nephrectomy as the treatment of choice, and mTOR inhibitors such as everolimus either as neoadjuvant or adjuvant targeted therapy can lead to a better clinical outcome. NGS to explore the mTOR signaling pathway may help us to better understand the pathogenesis and progression of EAML.

Renal epithelioid angiomyolipoma (eAML) is considered a malignant variant of angiomyolipoma (AML). From 2001 to 2016, a total of 570 patients were diagnosed with renal AML in Linko Chang Gung Memorial Hospital, Taiwan, including 23 cases of renal eAML. All 23 eAML cases were made up of at least 10% of epithelioid cells histologically. Three of these cases were found with multiple tumors. Two cases developed distant metastasis: one had mediastinal lymph nodes and bilateral lung metastasis; the other one had tumor recurrence over liver and retroperitoneum 1 year after radical nephrectomy. They were then divided into invasive (n = 5) and noninvasive (n = 18) groups according to their clinical behavior. The invasive group showed more severe nuclear atypia and higher rates in tumor necrosis. There was statistically no significance in relation to a patient's age, tumor size, and mitotic count between two groups. After conducting a series of studies, we suggest treating eAML with the guideline of renal cell carcinoma.