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    Association details:
    Biomarker:VHL mutation
    Cancer:Neuroendocrine Tumor
    Drug:Welireg (belzutifan) (HIF-2α inhibitor)
    Direction:Sensitive
    Evidence:
    Evidence Level:
    Sensitive: A1 - Approval
    Source:
    FDA
    Title:

    FDA Approves Merck’s Hypoxia-Inducible Factor-2 Alpha (HIF-2a) Inhibitor WELIREG™ (belzutifan) for the Treatment of Patients With Certain Types of Von Hippel-Lindau (VHL) Disease-Associated Tumors

    Published date:
    08/13/2021
    Excerpt:
    WELIREG is a hypoxia-inducible factor inhibitor indicated for treatment of adult patients with von Hippel-Lindau (VHL) disease who require therapy for associated renal cell carcinoma (RCC), central nervous system (CNS) hemangioblastomas, or pancreatic neuroendocrine tumors (pNET), not requiring immediate surgery.
    Evidence Level:
    Sensitive: C3 – Early Trials
    Source:
    J Clin Oncol
    Title:

    Phase 2 study of belzutifan (MK-6482), an oral hypoxia-inducible factor 2a (HIF-2a) inhibitor, for Von Hippel-Lindau (VHL) disease-associated clear cell renal cell carcinoma (ccRCC).

    Published date:
    05/28/2021
    Excerpt:
    For non-RCC tumors, ORR was 80% (16/20; 1 CR) in pNETs and 32% (16/50; 1 CR) in CNS hemangioblastomas...Belzutifan demonstrates clinical benefit and has a favorable safety profile in patients with VHL disease–associated ccRCC, pNETs, and hemangioblastomas.
    DOI:
    10.1200/JCO.2021.39.15_suppl.4555
    Trial ID:
    LITESPARK-004