The genetics of hereditary renal cancers

Renal cancers account for approximately 3.6% of all malignances represented in men and 2.6% in women. It is estimated that the increase in incidence of renal cancers is about 2-4% per year. The causes of kidney cancer are believed to be environmental and genetic. It is estimated that about 4% of renal cancer are “hereditary cancers”, thus due to high predisposition by constitutional genetic changes. The clinical hereditary forms of renal cancer are characterized by multifocal and generally bilateral tumours. Also vertical transmission has been observed. The age of diagnosis of patients with hereditary forms of renal cancer was younger than the age of patients with sporadic forms. Although to date there are over 20 syndromes described in which renal cell cancer may occur, only four syndromes with an unequivocal genetic predisposition to renal cell carcinoma have been identified: von Hippel-Lindau syndrome (VHL), hereditary clear cell carcinoma, hereditary papillary carcinoma (HPRC) and hereditary leiomyomatosis and papillary renal cell cancer syndrome (HLRCC). Most of them occur as a result of high monogenic predisposition. The advancement of molecular genetics will allow in the near future the identification of the polygenic background of cancer. The identification of a genetic predisposition to cancer has a great value because knowledge about the molecular basis of the carcinogenesis allows for a better understanding of the aetiology of the disease and also allows for the identification of individuals who are carriers of the mutations which means they are at risk of disease development.