The study of hereditary forms of kidney cancer has vastly increased our understanding of metabolic and genetic pathways involved in the development of both inherited and sporadic kidney cancers. The recognition that diverse molecular events drive different forms of kidney cancers has led to the preclinical and clinical development of specific pathway-directed strategies tailored to treat distinct subgroups of kidney cancer. Here, we describe the molecular mechanisms underlying the pathogenesis of several different types of hereditary renal cancers, review their clinical characteristics, and summarize the treatment strategies for the management of these cancers.
Keywords: Clear cell renal cell cancer; Fumarate hydratase; HLRCC; HPRC; Hereditary; Inherited; Kidney cancer; Metastatic; Papillary renal cell cancer; Renal cell cancer; Succinate dehydrogenase; Tuberous sclerosis; Tyrosine kinase inhibitors; Von Hippel-Lindau.