Not yet recruitingRare disease

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

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What is this trial about?

A plain-language summary of the goals, design and what participants do

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.

The research process

The trial runs in 5 steps – from screening to follow-up. Each step says what happens and what the team monitors.

Step 1
Informed consent and eligibility confirmation
After providing voluntary informed consent, your eligibility for the study will be confirmed.
This includes verification that you are at least 18 years old and have a performance status score of 0-2, which measures your ability to carry out daily activities.
If you are a woman who can become pregnant, a pregnancy test will be performed to ensure it is negative.
If you are in the Primary Cohort, confirmation of your Von-Hippel Lindau disease diagnosis through genetic testing showing a disease-causing variant will be verified.
If you are in the Secondary Cohort, confirmation of your diagnosis of hemangioblastoma (a type of blood vessel tumor), pheochromocytoma (a tumor of the adrenal gland), pancreatic neuroendocrine tumor (a tumor in the pancreas), or clear cell renal cell carcinoma (a type of kidney cancer) requiring surgery will be verified.
Step 2
Administration of imaging agent
You will receive an injection of Zr-DFO-girentuximab, which is a solution containing the active substance zirconium (89zr) girentuximab.
This medication will be administered through intravenous injection, which means it will be injected directly into a vein.
This imaging agent is designed to bind to Carbonic Anhydrase IX, a protein found on certain tumor cells, allowing them to be detected during scanning.
Step 3
Imaging scan
After receiving the injection, you will undergo a CAIX-PET scan, which is a specialized imaging test.
This scan is used to detect tumors by identifying areas where the imaging agent has attached to Carbonic Anhydrase IX on tumor cells.
The scan will help assess the presence and location of tumors related to Von-Hippel Lindau disease or the specific tumor types included in the study.
Step 4
Safety monitoring and contraception period
Your safety will be monitored throughout the study to evaluate any potential side effects from the imaging agent.
If you are a woman who can become pregnant, you must use highly effective contraception for 30 days following the injection.
If you are a man who can father a child, you must use highly effective contraception for 30 days following the injection.
Step 5
Follow-up and data collection
Information about the accuracy of the CAIX-PET scan in detecting your tumors will be collected.
The results will be used to evaluate how effective this imaging method is for identifying tumors in patients with Von-Hippel Lindau disease or the specific tumor types being studied.
Any safety information related to the imaging agent will also be recorded.

Who can join the trial?

7 criteria

You must voluntarily agree to participate in the study and sign an informed consent, which is a document explaining the study details and your rights
You must be 18 years old or older
Your ability to carry out daily activities must be good to moderate, rated as ECOG/WHO score 0-2, which is a scale doctors use to measure how well you can perform everyday tasks
If you are a woman who can become pregnant, you must have a negative pregnancy test and use very effective contraception, which means birth control methods, for 30 days after receiving the study medicine
If you are a man who can father a child, you must use very effective contraception, which means birth control methods, for 30 days after receiving the study medicine
For the main group: you must have a confirmed diagnosis of VHL disease that requires regular monitoring, confirmed by a genetic test showing a disease-causing change in your genes called a pathogenic variant
For the secondary group: you must have a diagnosis confirmed by examination or tissue testing of hemangioblastoma, which is a type of blood vessel tumor, pheochromocytoma, which is a tumor of the adrenal gland, pancreatic neuroendocrine tumor, which is a tumor in the pancreas, or clear cell renal cell carcinoma, which is a type of kidney cancer, and you need to have surgery for this condition

Who cannot join the trial?

8 criteria

Pregnancy or breastfeeding: Women who are pregnant or nursing a baby cannot take part in this study
Allergy to the study drug: You cannot participate if you have had an allergic reaction to the imaging agent or any of its ingredients in the past
Severe kidney problems: If your kidneys are not working well enough to filter waste from your blood, you cannot join this study
Other active cancer: You cannot participate if you have another type of cancer that is currently being treated or is not under control
Recent major surgery: If you have had a major operation within a certain time period before the study, you may not be eligible
Unable to undergo imaging scans: You cannot take part if you are unable to have the required scanning procedures, such as if you have metal implants that prevent safe scanning
Participation in another study: If you are currently taking part in another research study with an investigational drug, you may not be able to join this trial
Serious medical conditions: You cannot participate if you have other severe health problems that would make it unsafe for you to be in the study

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Verified sites

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Verified
Ospedale San Raffaele S.r.l.
Milan, Italy

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Investigated drugs

Carbonic Anhydrase IX is being studied as both a diagnostic tool and a potential treatment target in this trial. It is a protein that may help doctors identify and treat tumors in patients with Von-Hippel Lindau disease. The researchers want to see if this protein can be used to find tumors more easily and if it could be used to develop new treatments for this condition.

ZIRCONIUM (89ZR) GIRENTUXIMAB

What is already known about the treatment

Carbonic Anhydrase IX – Carbonic Anhydrase IX is a protein that is being studied as a potential diagnostic marker and treatment target in Von-Hippel Lindau disease, a genetic condition that causes tumors to grow in various parts of the body. This substance is currently in the research phase and is being explored in clinical trials to determine if it can help doctors identify and potentially treat tumors associated with this disease. The protein works at the molecular level by being overexpressed in certain tumor cells, making it a potential target for both imaging and therapy. This approach falls under the category of targeted therapy and molecular diagnostics, which aim to identify and treat disease by focusing on specific proteins or molecules involved in tumor growth.

Investigated diseases

Von Hippel-Lindau Disease – Von Hippel-Lindau disease is a rare inherited disorder that causes abnormal growth of blood vessels in various parts of the body. The condition results from mutations in a specific gene that normally helps control cell growth. People with this disease develop tumors and fluid-filled sacs called cysts in multiple organs, including the eyes, brain, spinal cord, kidneys, pancreas, and adrenal glands. These growths can appear at different times throughout a person's life, often beginning in young adulthood. The tumors are typically non-cancerous, but some can become cancerous, particularly in the kidneys. The disease affects different organs in different people, even within the same family, and symptoms depend on the size and location of the tumors.