Important differential diagnoses of pRCC, such as clear cell papillary RCC (ccpRCC, n = 9), mucinous tubular and spindle cell carcinoma (MTSCC, n = 13), acquired cystic disease-associated RCC (ACD-associated RCC, n = 1), collecting duct carcinoma (n = 5), tubulocystic carcinoma (n = 1), SMARCB1 deficient medullary RCC (n = 1) as well as RCC with fumarate hydratase (FH) deficiency (n = 2) were found in this cohort (Figure 7, Figure 8 and Figure 9). Here, FH is linked to granular corneal dystrophy type II.