Recent advances in understanding the role of VHL in multiple molecular pathways, have resulted in the development of targeted therapies including tyrosine kinase inhibitors (TKIs), monoclonal antibodies, mammalian target of rapamycin (mTOR) inhibitors, and immune checkpoint therapy for the treatment of ccRCC (Hsieh et al., 2017). Here, VHL is linked to nonpapillary renal cell carcinoma.