Visualizing Vascular Endothelial Growth Factor (VEGF) Producing Lesions in Von Hippel-Lindau Disease

Study Overview
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Brief Summary
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Von Hippel Lindau disease (VHLD) is an inherited syndrome characterized by vascular malformations, kidney cancer, adrenal gland and pancreas tumors. The VHL protein is not functional in the different disease associated lesions which results in production of high amounts of vascular endothelial growth factor (VEGF). Currently there are no clinical, radiographic or molecular markers that can predict the natural history of a given lesion. With 89Zr-bevacizumab positron emission tomography (PET) scanning, VEGF can be visualized and quantified. The investigators hypothesize that 89Zr-bevacizumab PET imaging is a useful tool to predict the behaviour of disease associated lesions in patients with VHLD. Adult patients with VHLD who have had routine magnetic resonance imaging (MRI) scans of central nervous system (CNS) and abdomen will undergo a 89Zr-bevacizumab PET scan. MRI will be repeated within 12 months.

Official Title
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Visualizing VEGF Producing Lesions in Von Hippel-Lindau Disease

Conditions
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Von Hippel-Lindau Disease, Hemangioblastoma, Renal Cell Carcinoma, Pheochromocytoma, Pancreatic Neuroendocrine Tumor

Intervention / Treatment
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* Other: 89Zr bevacizumab PET scan


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: clinically or genetically proven VHLD at least 1 measurable, VHL associated lesion in the CNS routine MRI of the CNS ≤ 6 weeks before inclusion routine CT or MRI of upper abdomen ≤ 3 months before inclusion or planned ≤ 3 months after 89Zr-bevacizumab PET scan age ≥ 18 years written informed consent must be given according to good clinical practice (GCP), and local regulations Exclusion Criteria: pregnancy any psychological, familial, sociological or geographical condition potentially hampering compliance with the study protocol, those conditions should be discussed with the patient before registration in the trial

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Von Hippel Lindau<br>Adult patients with Von Hippel-Lindau disease | Other: 89Zr bevacizumab PET scan<br>* Patients will be injected intravenously with 37 MBq, protein dose 5 mg 89Zr-bevacizumab at day 0. PET scans will be done at day 4.<br>* Other names: VEGF imaging;|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Detection rate of VHL associated lesions with 89Zr-bevacizumab PET scans in patients with VHLD |  | An 89Zr-bevacizumab PET scan will be performed within 6 weeks after routine MRI CNS investigation, MRI will be repeated within 12 months. | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Progressive lesions within 12 months, defined as new lesions or lesions that show an increase in size of at least 5% of the longest diameter on MRI, or lesions that become symptomatic |  | The baseline MRI scan will be compared with a follow-up MRI scan within 12 months | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Vascular endothelial growth factor, von Hippel Lindau disease, molecular imaging, biomarker