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Natural History of Plexiform in Neurofibromatosis Type I


N/A
N/A
N/A
Not Enrolling
Both
Neurofibroma, Plexiform, Neurofibromatosis Type I

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Trial Information

Natural History of Plexiform in Neurofibromatosis Type I


This is a four-year study, the overall objective of which is to set up a system that will
facilitate clinical trials of potential therapies for plexiform neurofibromas in NF1. These
tumors are unpredictable in terms of rate of growth and are difficult to measure. We will
set up a network of clinical centers and will follow the growth of plexiform neurofibromas
using volumetric MRIs. These centers will be supported by a tissue bank, in which tissue
samples will be made available to investigators throughout the world. In addition there
will be a database used to track clinical information about patients with NF1 and a standard
pathology review for any biopsy material. The first stage of this project will involve
setting up the infrastructure. Subject recruitment will begin in April 1999. The study will
then continue over a three-year period, during which patients will be present for clinical
assessments and MRIs. The protocol includes an algorithm for determination of the time for
repeat MRIs; clinical assessments will occur at 6 monthly intervals. The last six months of
the study will be devoted to data analysis.

Inclusion Criteria


- INCLUSION CRITERIA:

Subjects must fulfill two or more of the following diagnostic criteria for NF1:

Six or more cafe-au-lait macules (1. 1.5 cm or larger in postpubertal individuals; 2. 0.5
cm or larger in prepubertal individuals);

Two or more neurofibromas of any type or 1 or more plexiform neurofibroma;

Freckling in the axilla or groin;

Optic glioma (tumor of the optic pathway);

Two or more Lisch nodules (benign iris hamartomas);

A distinctive bony lesion (1. Dysplasia of the sphenoid bone; 2. Dysplasia or thinning of
long bone cortex);

A first degree relative with NF-1.

EXCLUSION CRITERIA:

Presence of metallic implant that will make the patient unable to have MRI studies.

Presence of a medical or psychological condition that will make the patient unable to
tolerate MRI studies or anesthesia (if needed).

Inability to image tumor or define tumor margins by MRI (which may be determined after the
initial study).

Failure to obtain initial MRI within 60 days of enrollment.

Previous radiation therapy to site of plexiform neurofibroma.

Surgery involving the plexiform neurofibroma (excluding biopsy) within a six month period
before enrollment.

Current antineoplastic therapy.

Entry of more than one member of the same family into the study is not permitted.

Type of Study:

Observational

Study Design:

N/A

Principal Investigator

Brigitte C Widemann, M.D.

Investigator Role:

Principal Investigator

Investigator Affiliation:

National Cancer Institute (NCI)

Authority:

United States: Federal Government

Study ID:

010027

NCT ID:

NCT00006435

Start Date:

October 2000

Completion Date:

Related Keywords:

  • Neurofibroma, Plexiform
  • Neurofibromatosis Type I
  • MRI Volumetric Analysis
  • Central Tissue Bank
  • Tumor Progression
  • Neurofibroma
  • Neurofibromatoses
  • Neurofibromatosis 1
  • Osteitis Fibrosa Cystica
  • Neurofibroma, Plexiform

Name

Location

National Institutes of Health Clinical Center, 9000 Rockville Pike Bethesda, Maryland  20892
Massachusetts General Hospital Boston, Massachusetts  02114-2617
Partners Center for Human Genetics Boston, Massachusetts  02115