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SEGA are diagnosed in 20% of patients with tuberous sclerosis complex (TSC). Their development is related to mutations of the tumor suppressor genes TSC1(located at 9q34) and TSC2 (located at 16p13.3), encoding, respectively, proteins named hamartin and tuberin. Such mutations result in hyperactivation of the PI3K/AKT/mTOR signaling pathway, which exhibits crosstalk with the MAPK/ERK signaling pathway [72]. Therefore, mTOR inhibitor rapamycin (sirolimus) and its analogues may play a role in the medical management of TSC-related tumors [72].