A report by Jonesch et al. showed the preliminary results of a phase II study (NCT 03401788) involving the treatment of 61 patients with germline VHL mutant, localized/nonmetastatic CCRCC, common lesions outside the kidney (non-RCC tumors such as hemangioblastomas (80%) and pancreatic lesions (50%)); about 28% of the patients displayed objective responses and about 87% of patients showed decrease in the size of target lesions [236]. Here, VHL is linked to nonpapillary renal cell carcinoma.