PEComas with TSC1/2 mutations or TFE3 rearrangements respond to mTORC1 inhibition [83,87,89], while IMTs harboring ALK, RET, PDGFRB, or NTRK rearrangements demonstrate marked responses to ALK or TRK inhibitors [90,106,112]. Here, TSC1 is linked to neoplasm with perivascular epithelioid cell differentiation.