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Association details:
| Biomarker: | VHL mutation |
|---|---|
| Cancer: | Clear Cell Renal Cell Carcinoma |
| Drug: | Welireg (belzutifan) (HIF-2α inhibitor) |
| Direction: | Sensitive |
Evidence:
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
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.
Source:
Title:
A Phase 2 Study of Belzutifan (PT2977, MK-6482) for the Treatment of Von Hippel Lindau (VHL) Disease-Associated Renal Cell Carcinoma (RCC) (MK-6482-004)
Excerpt:
...Has a diagnosis of von Hippel Lindau disease, based on a germline VHL alteration...
Trial ID:
Source:
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:
...22 confirmed responses (ORR, 36% [95% CI, 24-49]) and 7 (11%) unconfirmed responses (documented at 1 time point, to be confirmed at subsequent time point); all were PRs...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:
Source:
Title:
Phase II study of the oral HIF-2a inhibitor MK-6482 for Von Hippel-Lindau disease–associated renal cell carcinoma.
Published date:
05/25/2021
Excerpt:
MK-6482 showed promising efficacy and tolerability in pts with VHL-associated ccRCC and responses in other VHL-related lesions.
DOI:
10.1200/JCO.2020.38.15_suppl.5003
Trial ID:
Source:
Title:
Phase II study of the oral hypoxia-inducible factor 2a (HIF-2a) inhibitor MK-6482 for Von Hippel-Lindau (VHL) disease-associated clear cell renal cell carcinoma (ccRCC).
Published date:
02/08/2021
Excerpt:
Adults with germline VHL alterations, measurable, localized/non-metastatic ccRCC, no prior systemic anticancer therapy, and ECOG PS 0/1 received MK-6482 120 mg once daily until progression...There were 22 confirmed responses (ORR, 36% [95% CI, 24%-49%])...MK-6482 is an active and well-tolerated therapy for VHL disease–associated ccRCC
DOI:
10.1200/JCO.2021.39.6_suppl.333
Trial ID:
Source:
Title:
ORAL HIF-2a INHIBITOR MK-6482 FOR VON HIPPEL-LINDAU (VHL) DISEASE–ASSOCIATED CLEAR CELL RENAL CELL CARCINOMA: EVALUATION OF RCC AND NON-RCC DISEASE.
Published date:
11/20/2020
Excerpt:
For ccRCC, ORR was 36% (95% CI, 24-49%)...All responses were PRs...MK-6482 demonstrated durable efficacy in patients with VHL disease–associated ccRCC.
Trial ID:
Source:
Title:
LBA26 - Phase II study of the oral HIF-2a inhibitor MK-6482 for Von Hippel-Lindau (VHL) disease-associated clear cell renal cell carcinoma (ccRCC): Update on RCC and non-RCC disease
Published date:
09/19/2020
Excerpt:
Eligible patients (pts) were aged ≥18 yrs and had a VHL diagnosis based on germline VHL alteration, ≥1 measurable solid RCC tumor, no prior systemic anticancer therapy,...For ccRCC, ORR was 36% (95% CI, 24-49%) and an additional 7 (11%) unconfirmed responses (documented at single time point and pending confirmation at data cutoff) were reported by IRC; all responses were PRs. DOR in confirmed responses was not reached (NR; range, 12-62 wks). The PFS rate at 52 wks was 98% (95% CI, 89-100%).
Trial ID:
Source:
Title:
Merck’s Novel HIF-2a Inhibitor Showed an Objective Response Rate of Nearly 30% in Patients with von Hippel-Lindau (VHL) Disease-Associated Clear Cell Renal Cell Carcinoma
Published date:
05/13/2020
Excerpt:
The study enrolled adult patients with a pathogenic germline VHL variation, measurable localized or non-metastatic ccRCC...The primary endpoint was ORR of VHL-associated ccRCC tumors per RECIST v1.1 by independent radiology review....The results showed a confirmed ORR of 27.9% (n=17) (95% CI: 17.1-40.8); all responses were partial responses, and 43% of patients had stable disease.
Trial ID:
