Understanding and managing pancreatic tumors in patients with Von Hippel-Lindau disease
Official title: Evaluation of the Natural History and Management of Von Hippel-Lindau (VHL) Associated Pancreatic Neuroendocrine Tumors
Run by National Institutes of Health Clinical Center (CC) · NCT04074135 · Data updated 2026-11-12
This study is testing how pancreatic tumors behave in people with Von Hippel-Lindau disease to improve detection and management of these tumors.
Quick facts
| Phase | Phase 2 |
|---|---|
| Study type | Interventional |
| Ages | 12 Years and up |
| Sex | All |
| Enrollment | 740 (estimated) |
| Sponsor | National Institutes of Health Clinical Center (CC) NIH |
| Locations | 1 site (Bethesda, Maryland) |
| Status | Recruiting |
| Started | June 2, 2020 |
| Main results expected | January 30, 2036 |
| Study ends | July 1, 2036 |
| Registry record updated | October 16, 2026 |
| Trial ID | NCT04074135 on ClinicalTrials.gov |
Is this trial a fit for you?
✓Likely a good fit
Ideal candidates are individuals aged 12 and older diagnosed with Von Hippel-Lindau disease who have pancreatic manifestations.
✕Probably not a fit
Patients without a diagnosis of VHL or those unable to undergo serial non-invasive imaging may not benefit from this study.
A plain-English reading of the study's rules. Only the study team can confirm whether you qualify.
What this trial studies
This study aims to investigate the natural history of pancreatic neuroendocrine tumors associated with Von Hippel-Lindau (VHL) disease. Participants aged 12 and older with VHL will undergo comprehensive evaluations, including medical record reviews, imaging studies, and blood tests. The study will utilize advanced imaging techniques, specifically the 68-Gallium DOTATATE PET/CT scan, to enhance tumor detection and monitoring. By following these patients longitudinally, researchers hope to better understand the clinical spectrum of pancreatic manifestations in VHL.
Why it matters
Potential benefit: If successful, this study could lead to improved detection and management strategies for pancreatic tumors in VHL patients, potentially reducing life-threatening complications.
How similar studies have gone: While there have been studies on VHL and pancreatic tumors, the specific use of 68-Gallium DOTATATE PET/CT for this purpose is a novel approach.
Who can join: the full rules
Show full inclusion / exclusion criteria
* INCLUSION CRITIERIA:
1. Participants who have been diagnosed with VHL using the following criteria:
\-- Identification of a heterozygous germline pathogenic variant in VHL by molecular genetic testing.
or
\-- Clinical criteria
2. Participants with at least 1 pancreatic manifestation of VHL as documented on any non-invasive imaging study. These manifestations may include:
* Pancreatic cyst(s)
* Solid lesions suspicious for microcystic adenoma(s)
* Solid enhancing lesions suspicious for PNET(s)
* Any other solid lesion(s) of the pancreas
3. Age greater than or equal to 12 years.
4. Ability of participant to understand and the willingness to sign a written informed consent document.
EXCLUSION CRITERIA:
1\. Inability of participant to undergo serial non-invasive imaging.
Where it's running
-
National Institutes of Health Clinical Center
Bethesda, Maryland, United States Recruiting
Study contacts
- Principal investigator: Naris Nilubol, M.D. — National Cancer Institute (NCI)
- Study coordinator: Kristine J Villaruel
- Email: kristinejoy.villaruel@nih.gov
- Phone: (240) 858-7033
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How to take part
- Review the eligibility criteria above with your treating physician.
- Check the official listing on ClinicalTrials.gov for the latest contact details and recruitment status.
- Contact the study coordinator or principal investigator to ask for pre-screening. Pre-screening is free and never obligates you to enroll.