Molecular biology of clear cell renal carcinoma

Abstract

Kidney cancer is one of the ten most common cancers in the developed world [1]. Several histological variants of kidney cancer are recognized by pathologists including clear cell renal carcinoma, papillary (chromophil) renal carcinoma, chromophobic renal carcinoma, and oncocytoma [2]. The identification and study of rare families that are predisposed to kidney cancer led to the identifications of genes that, when mutated in the germline, confer an increased risk of the different histological variants of kidney cancer [3, 4]. For example, germline VHL mutations are linked to an increased risk of clear cell renal carcinoma, which is the most common form of kidney cancer. This chapter describes the molecular biology of clear cell renal carcinoma with an emphasis on the role of VHL in disease pathogenesis.