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Natural History and Management of Pancreatic Lesions in Von Hippel-Lindau Disease

This study is currently recruiting participants.
Verified April 2013 by National Institutes of Health Clinical Center (CC)
Sponsor:
Information provided by:
National Institutes of Health Clinical Center (CC)
ClinicalTrials.gov Identifier:
NCT00062166
First received: June 5, 2003
Last updated: May 1, 2013
Last verified: April 2013
History of Changes
  Purpose

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
Pancreatic Lesions

Study Type: Observational
Official Title: Evaluation of the Natural History and Management of Pancreatic Lesions Associated With Von Hippel-Lindau

Resource links provided by NLM:

U.S. FDA Resources

Further study details as provided by National Institutes of Health Clinical Center (CC):

Estimated Enrollment: 300
Study Start Date: April 2003
  Show Detailed Description

  Eligibility

Ages Eligible for Study:   12 Years and older
Genders Eligible for Study:   Both
Accepts Healthy Volunteers:   No
Criteria
  • INCLUSION CRITERIA:

Patients who have been diagnosed with VHL using the following criteria: either germ line analysis (12) or clinical criteria and a family history (8, 12) and who have at least 1 pancreatic manifestation of VHL as documented on any non-invasive imaging study. These manifestations include:

  1. Pancreatic cyst(s).
  2. Solid lesions suspicious for microcystic adenoma(s).
  3. Solid enhancing lesions suspicious for PNET(s).
  4. Any other solid lesion(s) of the pancreas.

Age greater than or equal to 12 years of age.

Patients must be willing to return to NIH for follow-up.

Patients/parent must be able to sign an informed consent.

EXCLUSION CRITERIA:

Patients unwilling to return to the NIH for follow-up.

Patients unwilling to undergo serial non-invasive imaging.

Patients without evidence of pancreatic lesions.

  Contacts and Locations
Please refer to this study by its ClinicalTrials.gov identifier: NCT00062166

Contacts
Contact: Erinn Hopkins (301) 496-6457 hopkinsem@mail.nih.gov
Contact: Electron Kebebew, M.D. (301) 496-5049 kebebewe@mail.nih.gov

Locations
United States, Maryland
National Institutes of Health Clinical Center, 9000 Rockville Pike Recruiting
Bethesda, Maryland, United States, 20892
Contact: Krisana Gesuwan, M.S.N.     301-402-4395     gesuwank2@mail.nih.gov    
Contact: Jean Ward     (301) 827-4988     jean.ward@nih.gov    
Sponsors and Collaborators
National Cancer Institute (NCI)
Investigators
Principal Investigator: Electron Kebebew, M.D. National Cancer Institute (NCI)
  More Information

Additional Information:
NIH Clinical Center Detailed Web Page  This link exits the ClinicalTrials.gov site

Publications:

ClinicalTrials.gov Identifier: NCT00062166     History of Changes
Obsolete Identifiers: NCT01444950
Other Study ID Numbers: 030145, 03-C-0145
Study First Received: June 5, 2003
Last Updated: May 1, 2013
Health Authority: United States: Federal Government

Keywords provided by National Institutes of Health Clinical Center (CC):
Imaging Studies
Rate of Tumor Growth
Familial Cancer Syndrome
Surgical Resection

Additional relevant MeSH terms:
Von Hippel-Lindau Disease
Neurocutaneous Syndromes
Nervous System Diseases
 Angiomatosis
Vascular Diseases
Cardiovascular Diseases

ClinicalTrials.gov processed this record on May 23, 2013