There was no difference in best DCR between patients treated with mTOR inhibitors and those that received other systemic therapies, both in the whole cohort (77.8% for n = 36 vs. 69.2% for n = 13, P = 0.70), in malignant PEComa only (63.3% for n = 22 vs. 66.7 for n = 22, P = 0.99), and in AML/LAM (P = 0.99; Supplementary Table S4). Here, MTOR is linked to neoplasm with perivascular epithelioid cell differentiation.