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

NCT00970970 | Completed

• 1 other identifier: METc2009.119
• Study Type: observational
• Target: 22
• Locations: 1 country
• Sites: 1

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 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.

Conditions

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

Keywords

Vascular endothelial growth factor; von Hippel Lindau disease; molecular imaging; biomarker

Outcome Measures

Primary Outcomes (1)

• 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 Outcomes (1)

• 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

Study Arms (1)

Von Hippel Lindau

Adult patients with Von Hippel-Lindau disease

Other: 89Zr bevacizumab PET scan

Interventions

89Zr bevacizumab PET scan OTHER

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.

Also known as: VEGF imaging

Von Hippel Lindau

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Patients will be selected from a tertiary referral center for Von Hippel-Lindau disease.

You may qualify if:

• clinically or genetically proven VHLD
• at least 1 measurable, VHL associated lesion in the CNS
• age ≥ 18 years
• written informed consent must be given according to good clinical practice (GCP), and local regulations

You may not qualify if:

• 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

Sponsors & Collaborators

• University Medical Center Groningen: lead
• VHL Alliance: collaborator

Study Sites (1)

University Medical Center Groningen

Groningen, 9700 RB, Netherlands

Location

Biospecimen

Retention: SAMPLES WITH DNA

Blood samples will be taken at day 1 and at the day of the MRI scan for analysis of VEGF pathway related biomarkers (such as plasma VEGF, PDGF, placental growth factor (PlGF), soluble VEGF receptors) and endothelial activation markers (such as Von Willebrand Factor (VWF), plasminogen activator inhibitor type 1 antigen (PAI-1), tissue-type plasminogen activator antigen (t-PA) and circulating endothelial cells (CECs)). DNA analysis for evaluation of polymorphisms in genes involved in angiogenesis is optional. In available biopsy or resection specimens, additional molecular staining of VEGF pathway related proteins will be performed (such as VEGF, VEGF receptors, HIF).

MeSH Terms

Conditions

von Hippel-Lindau Disease; Hemangioblastoma; Carcinoma, Renal Cell; Pheochromocytoma; Adenoma, Islet Cell

Condition Hierarchy (Ancestors)

Neurocutaneous Syndromes; Nervous System Diseases; Angiomatosis; Vascular Diseases; Cardiovascular Diseases; Ciliopathies; Abnormalities, Multiple; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Genetic Diseases, Inborn; Hemangioma, Capillary; Hemangioma; Neoplasms, Vascular Tissue; Neoplasms by Histologic Type; Neoplasms; Adenocarcinoma; Carcinoma; Neoplasms, Glandular and Epithelial; Kidney Neoplasms; Urologic Neoplasms; Urogenital Neoplasms; Neoplasms by Site; Female Urogenital Diseases; Female Urogenital Diseases and Pregnancy Complications; Urogenital Diseases; Kidney Diseases; Urologic Diseases; Male Urogenital Diseases; Paraganglioma; Neuroendocrine Tumors; Neuroectodermal Tumors; Neoplasms, Germ Cell and Embryonal; Neoplasms, Nerve Tissue; Adenoma; Pancreatic Neoplasms; Digestive System Neoplasms; Endocrine Gland Neoplasms; Digestive System Diseases; Pancreatic Diseases; Endocrine System Diseases

Study Officials

• Sjoukje Oosting, MD

  University Medical Center Groningen

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: September 2, 2009
• First Posted: September 3, 2009
• Study Start: September 1, 2009
• Primary Completion: November 1, 2012
• Study Completion: November 1, 2012
• Last Updated: May 6, 2024

Record last verified: 2024-05

Locations

NL (1)