Current understanding of the molecular mechanisms of kidney cancer: a primer for urologists

Renal cell carcinoma (RCC), the fifth leading malignant condition for men and tenth for women, accounts for 3% of all malignancies in Canada. It is a heterogeneous epithelial malignancy with different subtypes and varied tumour biology. Although most cases of RCC are sporadic, up to 4% of patients have an inherited predisposition for the disease. In this article, we review the current molecular genetics of the different subtypes in hereditary and sporadic RCC. Significant developments in understanding the underlying genetic basis of RCC over the last 2 decades are attributed to intensive research about rare inherited renal cancer syndromes and the identification of the genes responsible for them. Many of these genes are also found in sporadic RCC. Understanding the molecular mechanisms involved in the pathogenesis of RCC has aided the development of molecular-targeted drugs for this disease.