ABSTRACT
AIMS: A review of recent knowledge on heredital syndromes related to renal cell carcinoma. METHODS: Aim of this review was to summarize the recent knowledge of genetic syndromes associated with renal cell carcinoma. RESULTS: Summary of incidence and factors modulating risk of hereditary renal cell carcinoma development. CONCLUSIONS: Hereditary forms of RCC are relatively rare. Their study is beneficial in many ways. In individuals at a higher risk of a hereditary syndrome, the knowledge of hereditary forms may help to significantly decrease the impact of the hereditary disease. In the general population, knowledge acquired by the study of hereditary forms of RCC may in the future contribute to both diagnosis and treatment of sporadic tumours.