VHL and renal cell carcinoma: Like germline mutations in genes in the homologous recombination pathway and their association with hereditary breast and ovarian cancer; or germline mutations in mismatch repair genes and their association with colorectal cancer; germline mutations in genes associated with the hypoxia pathway (e.g., VHL, the SDHx genes, FH) appear to be associated with RCC and PGL/PCC.