The activity of the mTOR inhibitor nab-sirolimus in malignant PEComa was demonstrated in the AMPECT Phase 2 study, which showed an ORR of 38.7% (95% CI, 21.8 to 57.8), mPFS of 10.6 (95% CI, 5.5 to 41.2) months, and mDOR of 39.7 (95% CI, 6.5-NE) months [134,135]. Here, MTOR is linked to neoplasm with perivascular epithelioid cell differentiation.