RecruitingPhase 2NCT07167329

Real-World Effectiveness and Pharmacogenetics of Belzutifan in VHL Syndrome: The BELIEVE-VHL Trial

The BELIEVE-VHL Trial: A Real-world Longitudinal Study on Belzutifan's Effectiveness, Pharmacogenetics, and Pharmacoeconomics in Von Hippel-Lindau (VHL) Syndrome Using the HIF2α Inhibitor Belzutifan


Sponsor

José Claudio Casali da Rocha

Enrollment

100 participants

Start Date

Jan 1, 2024

Study Type

INTERVENTIONAL

Conditions

Summary

The BELIEVE-VHL Trial is a prospective real-life study designed to evaluate the therapeutic effects, benefits, and adverse effects of belzutifan, as well as the timing of treatment response and disease progression in patients with von Hippel-Lindau (VHL) syndrome.


Eligibility

Min Age: 14 Years

Inclusion Criteria7

  • Age ≥ 14 years.
  • Clinical or genetic confirmation of von Hippel-Lindau (VHL) syndrome.
  • Presence of measurable or progressive VHL-associated tumors, as defined by RECIST 1.1 or disease-specific imaging criteria.
  • ECOG performance status of 0-2.
  • Adequate bone marrow, hepatic, and renal function as defined by laboratory reference values.
  • Ability to swallow oral medication.
  • Provision of written informed consent prior to enrollment.

Exclusion Criteria13

  • Age \< 14 years.
  • Absence of a confirmed diagnosis of von Hippel-Lindau (VHL) syndrome.
  • Presence of an active malignancy outside the VHL tumor spectrum within the past 3 years, except for adequately treated basal or squamous cell carcinoma of the skin, cervical carcinoma in situ, or other malignancies considered cured for \>2 years.
  • Known hypersensitivity or allergic reaction to belzutifan or any excipient in the formulation.
  • History of severe or uncontrolled cardiovascular disease, including but not limited to unstable angina, myocardial infarction within the past 6 months, congestive heart failure requiring treatment, or uncontrolled hypertension.
  • Active infectious diseases, including HIV, hepatitis B, or hepatitis C.
  • Immunosuppressed status, whether due to underlying disease or ongoing therapy.
  • History of significant bleeding disorders, including bleeding diathesis, thrombocytopenia, or coagulopathy.
  • Radiotherapy administered within 4 weeks prior to study enrollment.
  • Major surgical procedure, including for VHL-related tumors, within 4 weeks prior to study enrollment, or immediate need for surgical intervention for tumor management.
  • Malabsorption secondary to prior gastrointestinal surgery or active gastrointestinal disease.
  • Current use of concomitant medications known to interact with belzutifan and significantly alter its bioavailability.
  • Anticipated low adherence to or planned interruption of belzutifan therapy.

Interventions

DRUGBelzutifan

Patients with Von Hippel-Lindau Syndrome presenting with lesions or neoplasms requiring treatment with oral Belzutifan.


Locations(1)

AC Camargo Cancer Center

São Paulo, São Paulo, Brazil

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NCT07167329


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