Namely, a translocation involving the TFE3 gene, a powerful stimulator of melanogenesis, in TFE3-rearranged PEComas, and the action of TFEB migrating to the nuclear site due to low levels of RagC-GDP, which prevents TFEB recruitment to MTORC1 and its subsequent phosphorylation in TSC-related PEComas [7]. Here, TFEB is linked to neoplasm with perivascular epithelioid cell differentiation.