Von Hippel-Lindau disease – European Clinical Trials Information Network

Study of Zirconium-89 Girentuximab as a Diagnostic Tool for Patients with Von-Hippel Lindau Disease

Wed, 29 Apr 2026 15:07:41 +0000

This study is looking at Von-Hippel Lindau disease, which is a genetic condition that can cause tumors to grow in different parts of the body. The study will also include people who have certain types of tumors that can occur with this condition, such as hemangioblastoma, pheochromocytoma, pancreatic neuroendocrine tumor, or clear cell renal cell carcinoma. The treatment being tested is called Zr-DFO-girentuximab, which is also known by its code name 89Zr-TLX250. This is a solution for injection that contains zirconium (89Zr) girentuximab and is given through a vein. The purpose of this study is to explore the role of Carbonic Anhydrase IX as a diagnostic and treatment target in Von-Hippel Lindau disease.

During the study, participants will receive a single injection of the study medication. The medication works by attaching to a specific protein called Carbonic Anhydrase IX that is often found on the surface of tumors related to this condition. After the injection, special imaging scans will be performed to see if the medication can help detect tumors in the body. This type of imaging is called CAIX-PET, which uses the study medication to create images that may show where tumors are located.

The study will look at how well this imaging method can find tumors in people with Von-Hippel Lindau disease and in people with certain types of tumors that can occur without the genetic condition. The study will also check the safety of using this imaging approach. Participants will be followed for a period of time after receiving the injection to monitor for any side effects and to complete the imaging procedures.

Study on Belzutifan for Treating Kidney Cancer in Patients with von Hippel-Lindau Disease

Wed, 29 Apr 2026 14:27:31 +0000

This clinical trial is focused on studying a condition known as von Hippel-Lindau disease-associated kidney cancer. This is a rare genetic disorder that can lead to the development of tumors in various parts of the body, including the kidneys. The trial is testing a medication called Belzutifan, also known by its code name MK-6482. This medication is taken orally in the form of a film-coated tablet.

The purpose of the study is to evaluate how effective Belzutifan is in treating kidney cancer associated with von Hippel-Lindau disease. Participants in the study will take the medication and be monitored over a period of time to see how their cancer responds. The study will look at various outcomes, such as how long the response to the treatment lasts and how quickly the treatment starts to work. The study will also track the time until any necessary surgery is performed and will evaluate the medication’s behavior in the body.

Participants will be required to take the medication as directed and attend regular check-ups to monitor their health and the progress of the treatment. The study aims to provide valuable information on the potential benefits of Belzutifan for individuals with von Hippel-Lindau disease-associated kidney cancer.

Study of Propranolol Treatment for Brain Hemangioblastomas in Patients with Von Hippel-Lindau Disease

Wed, 29 Apr 2026 14:23:00 +0000

This clinical trial focuses on von Hippel-Lindau disease, a rare genetic condition that causes tumors to grow in different parts of the body. Specifically, the study examines tumors called hemangioblastomas that develop in the brain and spinal cord (central nervous system). The study will test whether propranolol, a medication commonly used to treat high blood pressure, can help control the growth of these tumors.

The study will investigate how well propranolol works in treating brain and spinal cord tumors in people with von Hippel-Lindau disease. Participants will be divided into two groups, with one group receiving propranolol tablets and the other receiving a placebo. The treatment will continue for 24 months, during which patients will take the medication orally every day. The maximum daily dose of propranolol will be 120 mg.

Throughout the study, patients will have regular check-ups every 6 months, including MRI scans to monitor their tumors. Doctors will measure tumor size, growth rate, and other changes in the brain. They will also collect blood samples to measure certain proteins related to tumor growth and assess how patients are feeling and functioning in their daily activities. This will help determine if propranolol is effective in treating these tumors.