It has been suggested that TFE3 (transcription factor binding to IGHM enhancer 3) gene fusions and p53 gene mutation are involved in the genesis of malignant PEComa.[12,24] Moreover, the loss of TSC1 (chromosome 9q34) and TSC2 (chromosome 16p13.3), which are particularly associated with kidney AML,[25] was found to activate the pathway Rheb/mTOR/p70S6K (Ras homolog enriched in brain/ mammalian target of rapamycin/S6 kinase beta-1). This evidence concerns the gene RHEB and neoplasm with perivascular epithelioid cell differentiation.