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.
Additional locations may be listed on ClinicalTrials.gov for NCT04074135.
Locations matching your search criteria
United States
Maryland
Bethesda
National Institutes of Health Clinical CenterStatus: Active
Contact: National Cancer Institute Referral Office
Phone: 888-624-1937
Background:
- Patients with the von Hippel-Lindau (VHL) familial cancer syndrome demonstrate
manifestations in a variety of organs, including the pancreas. Pancreatic
manifestations can range from benign cysts and micro cystic adenomas to
neuroendocrine tumors of the pancreas which are capable of regional and distant
metastases. These neuroendocrine tumors can result in life-threatening
complications.
- This protocol is designed to identify VHL patients with pancreatic manifestations
and to follow these patients with serial imaging studies, germ line genetic
analysis, discovery of serum biomarkers, and novel imaging modalities such as
68-Gallium DOTATATE PET/CT scan.
Objective:
- To comprehensively and longitudinally evaluate the natural history of participants
with VHL pancreatic neuroendocrine tumors and cystic lesions, estimating and
defining their clinical spectrum.
Eligibility:
- Participants greater than or equal to 12 years of age diagnosed with VHL.
Design:
- Demographic data will be collected from the medical record and patient interview for
each participant. Data will be securely stored in a computerized database.
- Participants will be evaluated by the urology, neurosurgery, brain oncology and/or
ophthalmology personnel as indicated to rule out or manage other manifestations of
VHL such as hemangioblastoma, renal cell cancer, and pheochromocytoma.
- Anatomical and functional clinical imaging studies, research blood and urine sample
collection will be performed at each scheduled visit. Research 68-Gallium DOTATATE
PET/CT imaging studies in adult participants will be performed, no more than once
per year.
- Surgical resection of solid lesions of the pancreas will be recommended based on
previously published criteria.
- Based on our analysis of likelihood of tumor growth or risk of metastasis, data will
be analyzed every two years and appropriate revisions will be made to the surgical
management guidelines, if indicated by data analysis.
- Up to 700 participants may receive the research 68-Gallium DOTATATE PET/CT imaging
on this protocol.
Lead OrganizationNational Cancer Institute
Principal InvestigatorNaris Nilubol
