Some cases of AML, CCST, LAM, and CCMMT have been reported to be associated with tuberous sclerosis complex (TSC), a genetic disease caused by heterozygous mutations in the TSC1 (9q34) or TSC2 (16p13.3) genes, whereas only one of the reported cases of GI PEComas-NOS showed an association with TSC until now. Here, TSC1 is linked to tuberous sclerosis.