Hereditary Syndromes Associated with Kidney Tumors

Hereditary renal cell carcinomas (HRCC) comprise about 2–4% of all renal cell carcinomas (RCC). With understanding of molecular basis of RCC, 10 HRCC syndromes have been recognized. All of them exhibit an autosomal dominant inheritance pattern. More common and well-known HRCC syndromes are von Hippel–Lindau (VHL), hereditary papillary RCC, Birt–Hogg–Dube syndrome (BHD), hereditary leiomyomatosis RCC (HLRCC) syndrome, and tuberous sclerosis.