A significant proportion of PEComas occur in patients with tuberous sclerosis complex (a hereditary disease caused by mutations in the TSC1 or TSC2 genes), which encode proteins involved in the regulation of the mammalian target of rapamycin (mTOR) pathway, playing a critical role in cell growth and proliferation (30). Here, MTOR is linked to neoplasm with perivascular epithelioid cell differentiation.