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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)

Phase 2
Active, not recruiting
Conditions
VHL - Von Hippel-Lindau Syndrome
VHL Gene Mutation
VHL Syndrome
VHL Gene Inactivation
VHL-Associated Renal Cell Carcinoma
VHL-Associated Clear Cell Renal Cell Carcinoma
Interventions
Registration Number
NCT03401788
Lead Sponsor
Peloton Therapeutics, Inc., a subsidiary of Merck & Co., Inc. (Rahway, New Jersey USA)
Brief Summary

This study is designed to investigate belzutifan as a treatment for VHL disease associated RCC.

Detailed Description

This open-label Phase 2 study will evaluate the efficacy and safety of belzutifan in participants with VHL disease who have at least 1 measurable RCC tumor. Belzutifan will be administered orally and treatment will be continuous. Participants will be evaluated radiologically approximately 12 weeks after initiation of treatment and every 12 weeks thereafter while continuing in the study for a minimum of 3 years and then every 24 weeks or more frequently if clinically indicated. Changes in VHL disease-associated non-RCC tumors will also be evaluated.

Recruitment & Eligibility

Status
ACTIVE_NOT_RECRUITING
Sex
All
Target Recruitment
50
Inclusion Criteria
  • Has a diagnosis of von Hippel Lindau disease, based on a germline VHL alteration
  • Has at least 1 measurable solid RCC tumor and no RCC tumor that requires immediate surgical intervention. The diagnosis of RCC can be radiologic (histologic diagnosis not required). Participants may have VHL disease-associated tumors in other organ systems
Exclusion Criteria
  • Has received prior treatment with belzutifan or another HIF-2α inhibitor
  • Has had any systemic anti-cancer therapy (includes anti-vascular endothelial growth factor [VEGF] therapy or any systemic investigational anti-cancer agent)
  • Has an immediate need for surgical intervention for tumor treatment
  • Has evidence of metastatic disease on screening imaging

Study & Design

Study Type
INTERVENTIONAL
Study Design
SINGLE_GROUP
Arm && Interventions
GroupInterventionDescription
Open Label BelzutifanBelzutifanParticipants receive 120 mg belzutifan orally once daily. Participants may continue to receive belzutifan in the absence of unacceptable treatment related toxicity or unequivocal disease progression.
Primary Outcome Measures
NameTimeMethod
Objective Response Rate (ORR) in VHL Disease-Associated RCC TumorsUp to approximately 4 years

ORR is defined as the percentage of participants in the analysis population who have a best confirmed response of Complete Response (CR: Disappearance of all target lesions) or a Partial Response (PR: At least a 30% decrease in the sum of diameters of target lesions) per Response Evaluation Criteria in Solid Tumors version 1.1 (RECIST 1.1). ORR will be assessed by independent review committee (ICR) for the primary analysis.

Secondary Outcome Measures
NameTimeMethod
Duration of Response (DOR) in VHL Disease-Associated RCC TumorsUp to approximately 4 years

DOR is defined as the interval from the first documented evidence of a CR (disappearance of all target lesions) or a PR (at least a 30% decrease in the sum of diameters of target lesions) per RECIST 1.1 until Progressive Disease (PD) or death due to any cause, whichever occurs first, in participants demonstrating a best confirmed response of CR or PR. Per RECIST 1.1, PD is defined as at least a 20% increase in the sum of diameters of target lesions. In addition to the relative increase of 20%, the sum must also demonstrate an absolute increase of at least 5 mm. The appearance of one or more new lesions is also considered PD.

Time to Response (TTR) in VHL Disease-Associated RCC TumorsUp to approximately 4 years

TTR is defined as the interval from the start of study treatment to the first documentation of a response per RECIST 1.1, and calculated for participants with a best confirmed response of CR (disappearance of all target lesions) or PR (at least a 30% decrease in the sum of diameters of target lesions).

Progression-free Survival (PFS) in VHL Disease-Associated RCC TumorsUp to approximately 4 years

PFS is defined as the interval from the start of study treatment to the first documented PD or death from any cause, whichever occurs first. Per RECIST 1.1, PD is defined as at least a 20% increase in the sum of diameters of target lesions. In addition to the relative increase of 20%, the sum must also demonstrate an absolute increase of at least 5 mm. The appearance of one or more new lesions is also considered PD.

Time to Surgery (TTS) in VHL Disease-Associated RCC TumorsUp to approximately 4 years

TTS was defined as the interval from the start of study treatment to the date of surgery.

ORR in VHL Disease-Associated Non-RCC TumorsUp to approximately 4 years

ORR is defined as the percentage of participants in the analysis population who have a best confirmed response of CR (disappearance of all target lesions) or PR (at least a 30% decrease in the sum of diameters of target lesions) per RECIST 1.1.

DOR in VHL Disease-Associated Non-RCC TumorsUp to approximately 4 years

DOR is defined as the interval from the first documented evidence of a CR (disappearance of all target lesions) or a PR (at least a 30% decrease in the sum of diameters of target lesions) per RECIST 1.1 until PD or death due to any cause, whichever occurs first, in participants demonstrating a best confirmed response of CR or PR. Per RECIST 1.1, PD is defined as at least a 20% increase in the sum of diameters of target lesions. In addition to the relative increase of 20%, the sum must also demonstrate an absolute increase of at least 5 mm. The appearance of one or more new lesions is also considered PD.

TTR in VHL Disease-Associated Non-RCC TumorsUp to approximately 4 years

TTR is defined as the interval from the start of study treatment to the first documentation of a response per RECIST 1.1, and calculated for participants with a best confirmed response of CR (disappearance of all target lesions) or PR (at least a 30% decrease in the sum of diameters of target lesions).

PFS in VHL Disease-Associated Non-RCC TumorsUp to approximately 4 years

PFS is defined as the interval from the start of study treatment to the first documented PD or death from any cause, whichever occurs first. Per RECIST 1.1, PD is defined as at least a 20% increase in the sum of diameters of target lesions. In addition to the relative increase of 20%, the sum must also demonstrate an absolute increase of at least 5 mm. The appearance of one or more new lesions is also considered PD.

TTS in VHL Disease-Associated Non-RCC TumorsUp to approximately 4 years

TTS was defined as the interval from the start of study treatment to the date of surgery.

Number of Participants Experiencing an Adverse Event (AE)Up to approximately 4 years

An AE is any untoward medical occurrence in a participant regardless of its causal relationship to study treatment. An AE can be any unfavorable and unintended sign (including any clinically significant abnormal laboratory test result), symptom, or disease temporally associated with the use of the study drug, whether or not it is considered to be study drug related.

Number of Participants Experiencing a Serious Adverse Event (SAE)Up to approximately 4 years

An SAE is any AE occurring at any dose and regardless of causality that meets any of the following criteria: results in death, is life-threatening, requires inpatient hospitalization or prolongation of existing hospitalization, results in persistent or significant incapacity or substantial disruption of the ability to conduct normal life functions, is a congenital anomaly or birth defect in an offspring of a participant taking study drug, or is an important medical event.

Belzutifan Plasma ConcentrationWeeks 1 and 3: pre-dose, 2 and 5 hours post-dose, Week 5, 9, and 13: post-dose.

Blood samples for the determination of belzutifan concentration will be collected at pre-specified timepoints before and after treatment administration.

Belzutifan Metabolite Plasma ConcentrationWeeks 1 and 3: pre-dose, 2 and 5 hours post-dose, Week 5, 9, and 13: post-dose.

Blood samples for the determination of belzutifan metabolite concentration will be collected at pre-specified timepoints before and after treatment administration.

Trial Locations

Locations (11)

Massachusetts General Hospital

🇺🇸

Boston, Massachusetts, United States

University of Pittsburgh Medical Center

🇺🇸

Pittsburgh, Pennsylvania, United States

MD Anderson Cancer Center

🇺🇸

Houston, Texas, United States

Cambridge University Hospital

🇬🇧

Cambridge, United Kingdom

Huntsman Cancer Institute

🇺🇸

Salt Lake City, Utah, United States

Hospital Georges Pompidou

🇫🇷

Paris, France

University of Michigan

🇺🇸

Ann Arbor, Michigan, United States

Aarhus University Hospital

🇩🇰

Aarhus, Denmark

National Institutes of Health Clinical Center

🇺🇸

Bethesda, Maryland, United States

University of Pennsylvania Medical Center

🇺🇸

Philadelphia, Pennsylvania, United States

Vanderbilt Medical Center

🇺🇸

Nashville, Tennessee, United States

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