Mutations in TSC1 and TSC2 have been reported in a variety of neoplasms and benign tumors including pulmonary lymphangioleiomyomatosis (LAM), perivascular epitheloid cell tumors (PEComa), urothelial carcinomas, renal cell carcinoma and hepatocellular carcinomas [12]. The gene discussed is TSC1; the disease is renal cell carcinoma.