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

Related Clinical Trial
Propranolol and Von Hippel-Lindau Disease Retrospective Case Series of Trans-scleral Cryotherapy for Retinal Hemangioblastoma 68Ga-DOTA-TOC PET/CT in Imaging Participants With Neuroendocrine Tumors Treatment of Exudative and Vasogenic Chorioretinal Diseases Including Variants of AMD and Other CNV Related Maculopathy Study of Brain and Spinal Cord Tumor Growth and Cyst Development in Patients With Von Hippel Lindau Disease Assessment of Residual VHL Function in Tumors – Can it Predict the Patients’ Individual Course of Disease? Phase II Study of Vandetanib in Individuals With Kidney Cancer A Phase 2 Study of PT2977 for the Treatment of Von Hippel Lindau Disease-Associated Renal Cell Carcinoma Drivers of Hypoxia-induced Angiogenesis in Tumor Development Evaluation of 68Gallium-DOTATATE PET/CT for Detecting Neuroendocrine Tumors MyVHL: Patient Natural History Study Metabolic Mapping to Measure Retinal Metabolism Use of Tracking Devices to Locate Abnormalities During Invasive Procedures National Eye Institute Biorepository for Retinal Diseases Evaluation of Sunitinib Malate in Patients With Von Hippel-Lindau Syndrome (VHL) Who Have VHL Lesions to Follow Pazopanib in Von Hippel-Lindau (VHL) Syndrome 17AAG to Treat Kidney Tumors in Von Hippel-Lindau Disease Effect of Vorinostat on Nervous System Hemangioblastomas in Von Hippel-Lindau Disease (Missense Mutation Only) A Phase I/II Trial for Intravitreous Treatment of Severe Ocular Von Hippel-Lindau Disease Using a Combination of the PDGF Antagonist E10030 and the VEGF Antagonist Ranibizumab Ranibizumab Injections to Treat Retinal Tumors in Patients With Von Hippel-Lindau Syndrome Screening for Endolymphatic Sac Tumours (ELSTs) in Von Hippel-Lindau (vHL) Patients Bevacizumab (Avastin) in Unresectable/Recurrent Hemangioblastoma From Von-Hippel-Lindau Disease EYE001 to Treat Retinal Tumors in Patients With Von Hippel-Lindau Syndrome Von Hippel-Lindau Disease Genetic Epidemiology Study Treatment of Von Hippel-Lindau (VHL)-Related Hemangioblastoma With PTK787/ZK 222584 TKI 258 in Von Hippel-Lindau Syndrome (VHL) Study of Sunitinib in Patients With Von Hippel-Lindau (VHL) Disease Contrast-enhanced Ultrasound as a Screening Tool for Kidney Cancer in Patients With Von-Hippel Lindau Genetic Study to Identify Gene Mutations in Participants Previously Enrolled in Clinical Trial NCI-99-C-0053 Who Have Von Hippel-Lindau Syndrome or Are at Risk for Von Hippel-Lindau Syndrome PT2385 for the Treatment of Von Hippel-Lindau Disease-Associated Clear Cell Renal Cell Carcinoma Natural History and Management of Von Hippel-Lindau (VHL) Associated Pancreatic Neuroendocrine Tumors Visualizing Vascular Endothelial Growth Factor (VEGF) Producing Lesions in Von Hippel-Lindau Disease Natural History and Management of Pancreatic Lesions in Von Hippel-Lindau Disease Sunitinib Malate to Treat Advanced Eye Disease in Patients With Von Hippel-Lindau Syndrome

Brief Title

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

Official Title

Visualizing VEGF Producing Lesions in Von Hippel-Lindau Disease

Brief Summary

      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

      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.

Study Type


Primary Outcome

Detection rate of VHL associated lesions with 89Zr-bevacizumab PET scans in patients with VHLD

Secondary Outcome

 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


Von Hippel-Lindau Disease


89Zr bevacizumab PET scan

Study Arms / Comparison Groups

 Von Hippel Lindau
Description:  Adult patients with Von Hippel-Lindau disease


* Includes publications given by the data provider as well as publications identified by National Clinical Trials Identifier (NCT ID) in Medline.

Recruitment Information

Recruitment Status


Estimated Enrollment


Start Date

September 2009

Completion Date

November 2012

Primary Completion Date

November 2012

Eligibility Criteria

        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




18 Years - N/A

Accepts Healthy Volunteers



Sjoukje Oosting, MD, , 

Location Countries


Location Countries


Administrative Informations



Organization ID


Responsible Party

Principal Investigator

Study Sponsor

University Medical Center Groningen


 VHL Alliance

Study Sponsor

Sjoukje Oosting, MD, Principal Investigator, University Medical Center Groningen

Verification Date

November 2012