Notably, the combination of antibody-derived nivolumab ((NIVO), targeting anti-programmed death-1 (PD-1)), and ipilimumab ((IPI), targeting anti-cytotoxic T-lymphocyte-associated antigen 4 (CTLA-4)), has been established for mRCC patients with intermediate or poor risk as classified by the International Metastatic Renal Cell Carcinoma Database Consortium (IMDC) [3]. Here, CTLA4 is linked to hereditary clear cell renal cell carcinoma.