Management involves surgery, radiation, and/or systemic therapy, depending on the tumor type, location, invasion, and spread [4,7,10,12,13,14] Given that the vast majority of PEComas are driven by mTOR hyper-activation, the widely agreed upon systemic therapy is mTOR inhibition via everolimus, sirolimus, or temsirolimus [7]. This evidence concerns the gene MTOR and neoplasm with perivascular epithelioid cell differentiation.