Abstract

Although the incidence of renal neoplasms is not particularly high, their clinical presentation is often interesting. They tend to arise in association with familial neoplastic syndromes, i.e. renal cell carcinoma with von Hippel-Lindau disease. The cloning of the genes responsible for these hereditary pathological conditions revealed their involvement in sporadic renal cancers. Based on their molecular biological features, the classification of renal neoplasms was reassessed by the World Health Organization in 2004. In this chapter we discuss the relationships between the pathology and molecular biology of each histological type of renal neoplasm and the underlying molecular biological abnormalities.