The histopathologies of ccRCC (VHL disease or BAP1-associated tumor predisposition syndrome), SDH-mutated oncocytic RCC, and hereditary papillary RCC (constitutive activation of MET (type 1) or FH-mutated hereditary leiomyomatosis and RCC (type 2)) represents a single predominant histology, while TSC-mutated RCC represents heterogeneous components that show a striking contrast (Table 1) [55,86]. Here, SDHB is linked to nonpapillary renal cell carcinoma.