PEComas are related to the genetic alterations found in patients with TSC, an autosomal dominant genetic disease due to losses of TSC1 (9q34) or TSC2 (16p13.3) genes that may have a role in the regulation of the Rheb/mTOR/p70S6K pathway, which increase protein synthesis and consequently, cell growth [16]. Here, TSC1 is linked to tuberous sclerosis.