MTOR and tuberous sclerosis: Important clinical trials developed in recent years that have changed systemic treatments in TSC patients include EXIST-1 trial, completed in 2016 that demonstrated the efficacy and safety of Everolimus in SEGAs (Kingswood et al., 2014; Franz et al., 2016) and EXIST-2 trial that demonstrated the benefit of mTOR inhibitors for renal angiomyolipomas (AMLs) and resulted in Everolimus approval by the FDA for asymptomatic and growing renal AMLs larger than 3 cm (Bissler et al. 2013).