Each year there are >270 000 cases and 115 000 deaths from kidney cancer worldwide [1]. Although localized kidney cancer is most often treated successfully with surgery, patients who present with advanced disease have 2-yr survival of <20%. Kidney cancer is not a single disease; it is composed of a number of diseases, each of which has a different genetic cause, a clinical course that can be predicted on the basis of genotype, a different histology, and a unique response to therapy. Fifteen genes, including von Hippel-Lindau (VHL), met proto-oncogene (MET), folliculin (FLCN), fumarate hydratase (FH), succinate dehydrogenase (SDH), tuberous sclerosis 1 (TSC1), tuberous sclerosis 2 (TSC2), phosphatase and tensin homolog (PTEN), transcription factor binding to IGHM enhancer 3 (TFE3), transcription factor EB (TFEB), and microphthalmia-associated transcription factor (MITF), have been found to Cause or to be associated with the development of either sporadic or inherited forms of kidney cancer [2,3].
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