There is no established definitive treatment for PEComas; however, surgical resection is considered the most effective treatment approach. mTOR inhibitors (e.g., sirolimus, everolimus) and immune checkpoint inhibitors (e.g., pembrolizumab) are also considered for cases with malignant findings; however, evidence supporting their effectiveness remains limited [24-26]. This evidence concerns the gene MTOR and neoplasm with perivascular epithelioid cell differentiation.