Natural History and Management of Von Hippel-Lindau (VHL) Associated Pancreatic Neuroendocrine Tumors
VHL Pancreatic Neuroendocrine Tumors, Von Hippel-Lindau Disease, Neuroendocrine Tumors
About this trial
This is an interventional diagnostic trial for VHL Pancreatic Neuroendocrine Tumors focused on measuring Pancreatic tumors, 68-Gallium DOTATATE, MEN1 syndrome, NETest, PNET
Eligibility Criteria
INCLUSION CRITIERIA:
Subjects 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
Subjects 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
- Age greater than or equal to 12 years.
- Ability of subject to understand and the willingness to sign a written informed consent document.
EXCLUSION CRITERIA:
1. Inability of subject to undergo serial non-invasive imaging.
Sites / Locations
- National Institutes of Health Clinical CenterRecruiting
Arms of the Study
Arm 1
Arm 2
Experimental
No Intervention
1/ Arm 1
2/ Arm 2
Study natural history of VHL pancreatic neuroendocrine tumors with yearly 68-Gallium DOTATATE PET/CT research scans.
Study natural history of VHL pancreatic neuroendocrine tumors without research scans.