PBRM1 and nonpapillary renal cell carcinoma: The FDA has granted priority review for ipilimumab/nivolumab in intermediate and poor-risk mRCC (Abernathy, 2017), with RCC experts declaring this combination the new standard of care in the treatment of mRCC (Chustecka, 2017; Rini, 2017). Loss-of-function mutations in PBRM1 were discovered to confer clinical benefit to ccRCC patients treated with anti-PD-1 alone or in combination with anti-CTLA-4 therapies (Miao et al., 2018).