Natural History and Management of Pancreatic Lesions in Von Hippel-Lindau Disease
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|ClinicalTrials.gov Identifier: NCT00062166|
Recruitment Status : Completed
First Posted : June 6, 2003
Last Update Posted : December 2, 2017
Von Hippel-Lindau disease (VHL) is an inherited cancer syndrome. Patients are at risk for developing pancreatic cysts and tumors. These tumors are more aggressive in some people than in others. To learn more about this disease, its genetic cause and how best to treat it, this study will 1) identify patients with VHL who have pancreatic lesions; 2) examine the characteristics of the lesions and how fast they grow; 3) study how well imaging tests can reveal lesion characteristics that will help in diagnosis; and 4) perform genetic studies using blood and, when possible, tissue samples.
Patients 12 years of age and older with VHL involving the pancreas may be eligible for this study. Participants will undergo some or all of the following tests and procedures:
- Interviews with a cancer doctor, cancer nurses, and a surgeon (if surgery is recommended).
- Computed tomography (CT) scan of the abdomen, chest, or pelvis. This test uses x-rays to produce images of body tissues and organs in small sections.
- Magnetic resonance imaging (MRI) of the abdomen. This test uses radio waves and a strong magnetic field to produce images of body tissues and organs.
- Ultrasound of the abdomen. This test uses sound waves to create images body tissues and organs.
- Blood tests for routine laboratory chemistries, for tests specific to the pancreas, and for genetic studies
- 24-hour urine studies
After the tests are completed, the doctor will discuss the results with the patient. Patients with a pancreatic tumor that requires surgery will be offered the option of an operation to remove as much tumor as possible. Patients with lesions that are not appropriate for surgery will be asked to return to NIH for scans and x-rays every year to monitor growth of the lesions. If surgery should become advisable in the future, the option will be discussed at that time. Patients with pancreatic cysts will be asked to return to NIH every 2 years for scans and x-rays to monitor their condition.
|Condition or disease|
|Von Hippel-Lindau Disease|
Show Detailed Description
|Study Type :||Observational|
|Actual Enrollment :||340 participants|
|Official Title:||Evaluation of the Natural History and Management of Pancreatic Lesions Associated With Von Hippel-Lindau|
|Study Start Date :||April 11, 2003|
|Study Completion Date :||November 29, 2017|
- To identify patients with VHL having pancreatic lesions defined by simple cysts, microcystic adenomas, neuroendocrine tumors and other solid lesions of the pancreas. [ Time Frame: Ongoing ]
- To obtain blood samples from patients to determine VHL mutation status and subtype the mutations for potential correlation with disease severity. [ Time Frame: Ongoing ]
- When possible, to obtain tissue from pancreatic lesions for genetic analysis including CGH, tissue proteomics, and cDNA microarray analysis. [ Time Frame: Ongoing ]
To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.
Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT00062166
|United States, Maryland|
|National Institutes of Health Clinical Center, 9000 Rockville Pike|
|Bethesda, Maryland, United States, 20892|
|Principal Investigator:||Electron Kebebew, M.D.||National Cancer Institute (NCI)|