Recruiting
Phase 2

Observational Study

Sponsor:

National Cancer Institute (NCI)

Code:

NCT04074135

Conditions

VHL Pancreatic Neuroendocrine Tumors

Von Hippel-Lindau Disease

Neuroendocrine Tumors

Eligibility Criteria

Sex: All

Age: 12+

Healthy Volunteers: Not accepted

Interventions

68-Gallium DOTATATE

Study Details

Brief summary:

Background:

People with von Hippel-Lindau (VHL) can have problems with a variety of organs, such as the pancreas. The disease can cause tumors of the pancreas. This can result in life-threatening complications. Researchers want to learn more about these pancreatic tumors and how to better detect them. This may help them design better future treatment and care for people with VHL disease.

Objective:

To better understand VHL disease that affects the pancreas and to test whether adding a certain type of scan (68-Gallium DOTATATE PET/CT) can further detect tumors.

Eligibility:

People ages 12 and older with VHL that causes tumors and cysts to grow in the pancreas

Design:

Participants will be screened with their medical records and imaging studies.

Participants will have an initial evaluation:

Participants will have their body examined by different doctors. This will depend on what types of symptoms they have.

Participants will have blood and urine tests

Participants will have images made of their body using one or more machines: They made have a CT or PET/CT scan in which they lie on a table that moves through a big ring. They may have an MRI in which they lie on a table that moves into a big tube. They may have an ultrasound that uses a small stick that produces sound waves to look at the body.

After the first visit, participants will be asked to return to the NIH. Some of the tests performed at the first visit will be repeated. Depending on their disease status, visits will be once a year or every 2 years for life.

Conditions

VHL Pancreatic Neuroendocrine Tumors

Von Hippel-Lindau Disease

Neuroendocrine Tumors

Study ID

NCT04074135

Start date

Jun 2, 2020

Status verified date

Apr 10, 2026

Completion date

Jul 1, 2036

Anticipated

Primary completion date

Jan 30, 2036

Anticipated

Eligibility Criteria

Eligibility Criteria

Sex: All

Age: 12+

Healthy Volunteers: Not accepted

  • 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.

Study Design

Enrollment

740 participants

Anticipated

Allocation

Non randomized

Intervention Model

Parallel Assignment

Primary purpose

Diagnostic

Interventions and Outcome Measures

Arms

experimental: 1/ Arm 1

Study natural history of VHL pancreatic neuroendocrine tumors with yearly 68-Gallium DOTATATE PET/CT research scans.

no intervention: 2/ Arm 2

Study natural history of VHL pancreatic neuroendocrine tumors without research scans.

Interventions

68-Gallium DOTATATE

68-Gallium DOTATATE, to be used in yearly PET/CT research scans

Primary outcome measure

  • Characterization of the natural and clinical histories of VHL pancreatic neuroendocrine tumors and cystic lesions [ Time Frame: 15 years ]

Central Contacts and Locations

Central contacts

Kristine J Villaruel

(240) 858-7033kristinejoy.villaruel@nih.gov

Naris Nilubol, M.D.

(240) 760-6154niluboln@mail.nih.gov

Locations

National Institutes of Health Clinical Center

Recruiting

Bethesda, Maryland, United States, 20892

Contacts

For more information at the NIH Clinical Center contact National Cancer Institute Referral Office

888-624-1937

More Information

Sponsor

National Cancer Institute (NCI)

Last update posted

Apr 20, 2026

Last verified

Apr 10, 2026

Keywords

  • Pancreatic tumors
  • 68-Gallium DOTATATE
  • MEN1 syndrome
  • NETest
  • PNET

Trial information was received from ClinicalTrials.gov and was last updated on 2026-09-10. This information was provided to ClinicalTrials.gov by National Cancer Institute (NCI) on 2026-04-20.