These mutations lead to the activation of the mammalian target of rapamycin pathway.[9–11] In addition, p53 mutations can be seen frequently in TSC-2 mutant malignant PEComas.[9,12] Infrequently, TSC-2 non-mutant PEComas shelter translocations containing transcription factor binding to IGHM Enhancer 3.[9]. This evidence concerns the gene TSC2 and neoplasm with perivascular epithelioid cell differentiation.