Although other mTOR inhibitors have been used off-label for treatment of advanced malignant PEComa, to our knowledge, the AMPECT study is the first prospective study in this disease and provides evidence that nab-sirolimus may offer an important benefit in a rare and aggressive sarcoma for which there are no approved therapies. This evidence concerns the gene MTOR and neoplasm with perivascular epithelioid cell differentiation.