Protocol Number: 00-N-0140

Title:

A Prospective Natural History Study of VHL Patients with CNS Hemangioblastomas

Number:

00-N-0140

Summary:

The purpose of this study is to learn more about the growth of brain and spinal cord tumors and cysts that develop in association with them in patients with von Hippel-Lindau disease. It will examine how fast the tumors grow and try to determine what factors (for example, puberty , pregnancy, menopause, blood proteins, etc.) affect their growth.

Patients between the ages of 8 and 75 years who are enrolled in NIH's study of von Hippel-Lindau disease may be eligible for this 5-year study. Participants will have magnetic resonance imaging (MRI) of the brain and spinal cord and a thorough neurological history and examination at the start of the study. A blood sample will be taken for analysis of factors (hormones or other proteins) that may predict tumor growth. Follow-up clinic visits every 6 months will include a physical and neurological examination, blood tests, and MRI scans of the brain and spine. If symptoms or tumor growth requires more frequent follow-up, scans will be done at 3-month intervals.

Surgical removal of brain and spinal cord tumors is currently the treatment of choice when these lesions cause neurological problems. A better understanding of which tumors are likely to grow and which will remain stable may help guide physicians in treatment decisions and avoid unnecessary procedures.

Sponsoring Institute:

National Institute of Neurological Disorders and Stroke (NINDS)

Recruitment Detail

Type: Active Accrual Of New Subjects

Gender: Male & Female

Referral Letter Required: No

Population Exclusion(s): None

Eligibility Criteria:

INCLUSION CRITERIA:

Patients will be recruited from the NIH von Hippel-Lindau (VHL) clinic at the National Institutes of Health (NIH). All patients will have the diagnosis of von Hippel-Lindau disease confirmed using existing established criteria and meet the following requirements:

Consenting men and women between the ages of 18 and 75, inclusive.

Males and females between the ages of 8-18 whose parents/guardians can provide permission.

Presence of one or more cerebral, cerebellar or spinal cord hemangioblastomas on screening MRI.

Provide written informed consent prior to participation in the trial.

Karnofsky Performance Scale Score greater than or equal to 60.

Capacity to undergo MRI scanning.

EXCLUSION CRITERIA:

Clinically unstable condition.

Karnofsky Performance Scale Score less than or equal to 60.

Being treated by any chemotherapy, immunotherapy, or steroids.

Allergy to CT or MRI contrast agents.

Contraindication to MR scanning such as surgery that involves metal clips or wires which might be expected to cause tissue damage or produce image artifacts.

Special Instructions: Currently Not Provided

Keywords:

Brain

Brainstem

Cyst

Magnetic Resonance Imaging

Spinal Cord

Recruitment Keywords:

von Hippel-Lindau

Conditions:

Hemangioblastoma

Hippel Lindau Disease

Investigational Drug(s):

None

Investigational Device(s):

None

Contacts:

Patient Recruitment and Public Liaison Office
Building 61
10 Cloister Court
Bethesda, Maryland 20892-4754
Toll Free: 1-800-411-1222
TTY: 301-594-9774 (local),1-866-411-1010 (toll free)
Fax: 301-480-9793

Electronic Mail:prpl@mail.cc.nih.gov

Citations:

Treatment of hemangioblastomas in von Hippel-Lindau disease with linear accelerator-based radiosurgery

Radiosurgical treatment of a hemagioblastoma: case report

The cyberknife: a frameless robotic system for radiosurgery

• Questions about participating in a study, please contact the Patient Recruitment and Public Liaison Office, CC.
• Technical questions regarding the Clinical Center web site, please contact the Department of Networks and Applications, CC.

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Warren Grant Magnuson Clinical Center (CC)
National Institutes of Health (NIH)
Bethesda, Maryland 20892. Last update: 10/22/2004