In addition, many cases of syndromic and sporadic PEComas have loss-of-function mutations inTSC1orTSC2genes, resulting in activation of the mammalian target of rapamycin (mTOR) pathway, which acts as a target of mTOR inhibitors, which can be used in the treatment of these cases.13 RecurrentRAD51Bgene fusions have been reported only in uterine PEComas.13 Infrequently, PEComas can be associated with TFE3 translocations. Here, TFE3 is linked to neoplasm with perivascular epithelioid cell differentiation.