PEComas can be related to genetic alterations of tuberous sclerosis complex [TSC], an autosomal dominant disease due to loss of TSC1 or TSC2 genes involved in the regulation of the Phosphatidylinositol 3-Kinases [PI3K]/AKT/Mammalian Target of Rapamycin [mTOR] signaling pathway (Kwiatkowski, 2003). The gene discussed is TSC2; the disease is neoplasm with perivascular epithelioid cell differentiation.