Tuberous sclerosis complex (TSC)-associated kidney cancer is another autosomal dominant syndrome characterized by different renal tumors (for example, angiomyolipoma and its malignant variant epithelioid angiomyolipoma) and caused by alterations in two genes, TSC1 and TSC2, that induce the constitutive activation of the LKB1/AMPK/TSC/mTOR pathway [74]. Here, TSC1 is linked to kidney cancer.