Renal cell carcinoma (RCC) is the sixth most common cancer in men and the eighth most common in women.[sup.1] In Canada there were 5900 new diagnoses and 1740 deaths from RCC in 2013.[sup.2] Most are sporadic; however between 4% and 8% can be classified as hereditary.[sup.3,4] Some authors suggest that the true incidence of hereditary RCC (HRCC) may be much higher, but current estimates suffer from under diagnosis and underreporting.[sup.5,6] Several hereditary syndromes have been identified, the most common of which are Von Hippel-Lindau (VHL), hereditary papillary RCC (HPRCC
), hereditary leiomyomatosis and RCC (HLRCC), Birt-Hogg-Dube syndrome (BHD), and tuberous sclerosis complex (TSC).