TSC1 and renal cell carcinoma: Examples include a study of rapalog-treated metastatic RCC patients, which identified an enrichment for MTOR, TSC1 and TSC2 mutations in responders [376], a report of a single exceptional responder to pazopanib and everolimus with urothelial carcinoma who was found to have two activating mutations in MTOR [377], and two studies of patients with perivascular epithelioid cell tumors (PEComas) who responded to sirolimus, some of which demonstrated loss of TSC1/TSC2 or TSC2 aberrations [378, 379].