In this chapter, we discuss the development of the parenchymal kidney and its anatomic and histologic compartments. The chapter concentrates on renal cell carcinoma, beginning with renal cell carcinomas with molecular abnormalities and those associated with hereditary syndromes. The remainder of the chapter discusses mesenchymal lesions, familial syndromes, and renal cell carcinomas without known molecular drivers. Renal malignancies can be divided into a wide breadth of histologic subtypes, and the emerging molecular underpinnings pose an evolving challenge in disease categorization and prognostication. While early research relied on the link between familial genetic syndromes and renal cell carcinoma, the direction has shifted to molecular and environmental drivers of sporadic renal cell carcinomas.
