In advanced disease, mTOR inhibitors such as everolimus, which is used in all NET locations (as second-line treatment), could be of particular interest in TSC patients; however, no studies to date have demonstrated specific efficacy in those patients, except for one case with metastatic pNET and a TSC2 germline mutation that showed partial response shortly after the introduction of everolimus. The gene discussed is TSC2; the disease is tuberous sclerosis.