CAIX-targeted PET imaging to find tumors in people with von Hippel‑Lindau (VHL) disease

Is this trial a fit for you?

A plain-English reading of the study's rules. Only the study team can confirm whether you qualify.

What this trial studies

This is a phase 2, single‑arm, non‑randomized study in which participants receive one injection of [89Zr]Zr‑DFO‑girentuximab followed by hybrid PET/CT imaging to visualize carbonic anhydrase IX (CAIX) expression. The imaging results will be compared to standard-of-care references such as MRI and pathology from surgery to measure sensitivity and diagnostic accuracy. The protocol includes a primary cohort of adults with genetically confirmed VHL under surveillance and a secondary cohort of patients with hemangioblastoma, pheochromocytoma, pancreatic neuroendocrine tumor, or clear‑cell renal cell carcinoma scheduled for surgery. The study also models a theranostic approach by substituting the diagnostic isotope’s decay with therapeutic isotopes in dosimetry simulations to estimate potential therapeutic targeting.

Why it matters

Who can join: the full rules

Inclusion Criteria:

* Voluntarily given informed consent
* Age ≥18 years old
* Performance Status ECOG/WHO score 0-2
* For females of reproductive potential, negative pregnancy test and use of highly effective contraception for 30 days following IMP administration
* For males of reproductive potential, use of highly effective contraception for 30 days following IMP administration.

And, for the primary cohort:

* Diagnosis of VHL disease requiring surveillance following confirmation of pathogenic variant at genetic test

Alternatively, for the secondary cohort:

\- Clinical and/or pathological diagnosis of hemangioblastoma, pheochromocytoma, pancreatic neuroendocrine tumor or clear cell renal cell carcinoma requiring surgery.

Exclusion Criteria:

* Performance Status ECOG/WHO score \>2
* Women who are pregnant or breastfeeding or are planning pregnancy during the study
* Men who are planning fatherhood during the study
* Exposure to any murine or chimeric antibodies within 5 years prior to the planned IMP administration
* Exposure to any experimental diagnostic or therapeutic drug within 30 days from the planned IMP administration
* Surgery, biopsy, ablative procedure, radiotherapy or any other local treatment for any primary tumor within 4 weeks prior to the planned IMP administration
* Exposure to any systemic agent within 4 weeks prior to the planned IMP administration or in case of continuing adverse effects with grade \>1 from such therapy
* Current exposure to systemic agents or scheduled therapy in the next 6 months following the planned IMP administration
* Serious non-malignant disease (e.g. psychiatric, infectious, autoimmune or metabolic) that may interfere with the objectives of the study or within the safety of compliance of the subjects as judged by the Investigator
* Known hypersensitivity to \[89Zr\]Zr-DFO-Girentuximab or DFO (Desferrioxamine)
* Severe chronic kidney disease with glomerular filtration rate ≤ 30 mL/min/1.73m2
* Other vulnerable categories than rare disease (e.g, being in detention)

Where it's running

• IRCCS Ospedale San Raffaele
  Milan, Italia, Italy Recruiting

Study contacts

• Study coordinator: Alessandro Larcher
• Email: larcher.alessandro@hsr.it
• Phone: +39 02.2643.4121

Similar studies

Other studies for the same condition or treatment. Recruiting studies are listed first.

• Belzutifan for VHL-associated tumors: patient registry

  This registry collects real-world data on adults with VHL who choose belzutifan to see how well it works and how safe it is over time.

  Recruiting Chinese University of Hong Kong · Shatin · NCT07554508

• Biorepository for studying retinal diseases

  This study is collecting health information and samples from people with and without eye diseases to see how these conditions develop and respond to treatments.

  Recruiting National Institutes of Health Clinical Center (CC) · Bethesda, Maryland · NCT01496625

• CAIX-targeted 18F-T2 PET/CT imaging in solid tumors

  This study tests a CAIX-specific radioactive tracer to see if PET/CT can help visualize solid tumors and checks safety and radiation exposure in adults with certain cancers.

  Recruiting Peking University First Hospital · Beijing, Beijing Municipality · NCT07557225

• Extension program for people previously treated with belzutifan (MK-6482)

  This extension lets people with advanced solid tumors or VHL-related tumors who were already taking belzutifan in another study continue the drug so researchers can see how long they live and watch for long-term effects.

  Recruiting at 50 sites Phase 3 Merck Sharp & Dohme LLC · Boston, Massachusetts and 56 other locations · NCT07405164

• Real-world belzutifan outcomes and pharmacogenetics in von Hippel-Lindau syndrome

  This study will test belzutifan in people aged 14 and older with VHL syndrome to see how well it controls VHL-associated tumors, what side effects occur, and how genetic factors affect response.

  Recruiting Phase 2 AC Camargo Cancer Center · São Paulo, São Paulo · NCT07167329

• Registry for Rare Diseases

  This study is creating a detailed registry to collect information on rare diseases like amyloidosis and sarcoidosis to help improve how they are diagnosed and treated.

  Recruiting Hospital Italiano de Buenos Aires · Buenos Aires, Buenos Aires · NCT06573723

How to take part

1. Review the eligibility criteria above with your treating physician.
2. Check the official listing on ClinicalTrials.gov for the latest contact details and recruitment status.
3. Contact the study coordinator or principal investigator to ask for pre-screening. Pre-screening is free and never obligates you to enroll.