Novel Treatment for von Hippel–Lindau–Related Clear Cell Renal Cancer

The HIF-2α inhibitor belzutifan showed promising results in a phase 2 trial.

Patients with von Hippel–Lindau (VHL) disease, which is caused by germline pathogenic variations in the VHL gene, have a high incidence of clear cell renal cancer (ccRC) in addition to a myriad of extra-renal manifestations, including hemangioblastomas of the central nervous system (CNS) and pancreatic neuroendocrine tumors. Hypoxia-inducible factor 2α (HIF-2α) is a transcription factor known to drive the progression of ccRC.

Investigators conducted an industry-sponsored multinational phase 2 trial of a novel HIF-2α inhibitor, belzutifan, in patients with VHL-related ccRC. Eligible patients were required to have good ECOG performance status and at least one measurable site of disease. Patients received belzutifan orally at 120 mg daily. The …

Reviewing Author

Robert Dreicer, MD, MS, MACP, FASCO

Disclosures

Consultant/Advisory BoardAstellas; Aveo; Bayer; Gilead; Merck; Pfizer; Propella; Macrogenics

Editorial BoardsCurrent Urology Reports

Robert Dreicer, MD, MS, MACP, FASCO

Disclosures

Consultant/Advisory BoardAstellas; Aveo; Bayer; Gilead; Merck; Pfizer; Propella; Macrogenics

Editorial BoardsCurrent Urology Reports

Novel Treatment for von Hippel–Lindau–Related Clear Cell Renal Cancer

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Reviewing Author