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The Hereditary Renal Cancer Syndromes

Although the majority of renal cell carcinomas (RCC) are sporadic, hereditary types of kidney cancer such as Von Hippel-Lindau (VHL) disease and hereditary papillary renal carcinoma syndromes are thought to account for approximately 4% of kidney cancers. A germline mutation seems to predispose to the development of specific histologic types of RCC. Hereditary kidney cancers are bilateral multifocal kidney tumors that, in some instances, occur at a younger age than do sporadic renal cancers. A better knowledge of the genetic basis of renal carcinogenesis has been useful in identifying genetic causes of renal tumorigenesis, such as inactivation of the VHL tumor suppressor gene in clear cell carcinoma. Moreover, the genes responsible for hereditary renal cancer syndromes seems to play a role in the development of sporadic counterparts. Knowledge of genetic cancer syndromes may allow clinicians to screen and counsel family members, identify those patients at risk for multiple cancer development, and give the chance to intervene therapeutically when cancers are still treatable by identifying localized tumors through early screening. This article reviews the current status and recent advances in the field of familial kidney cancer syndromes.