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The purpose of this study is to document the rate of growth of plexiform neurofibromas over a 3-year period. Plexiform neurofibromas are a type of benign tumor seen in patients with neurofibromatosis type 1. The growth of these tumors is unpredictable and the factors that influence their growth are largely unknown.
Children and adults with plexiform neurofibromas may be eligible for this study. At the first clinic visit a blood sample of about 5 to 10 milliliters (1 to 2 teaspoons) will be drawn. DNA from the sample will be stored for possible future study of changes in the NF1 gene or other genes that may affect the behavior of neurofibromas. The serum may be used to test for substances in the blood that cause neurofibromas to grow.
Every 6 months patients will have a standard physical examination. In addition, if the plexiform neurofibroma is visible on the skin surface, it will be measured with a tape measure or ruler and photographed. Photographs will include only the part of the body affected by the neurofibroma, and they will be kept in a confidential file without any patient identifier.
Patients will have magnetic resonance imaging (MRI) scanning of the plexiform neurofibroma three times during the study-at enrollment, at 1 year and at 3 years. Scans will be done more often if the plexiform neurofibroma appears to be growing rapidly or if other problems are noted.
Surgery will not be done as part of this study. If, however, surgery is performed as part of standard medical care and tumor tissue is available that is not needed for clinical purposes, a sample will be stored for possible distribution to investigators conducting research on neurofibromatosis.
Patients who do not wish to contribute a blood and/or tumor sample, may still participate in the study.
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.
National Cancer Institute (NCI)
Active, not recruiting
National Institutes of Health Clinical Center (CC)
Published on BioPortfolio: 2014-08-27T03:57:18-0400
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A type of neurofibroma manifesting as a diffuse overgrowth of subcutaneous tissue, usually involving the face, scalp, neck, and chest but occasionally occurring in the abdomen or pelvis. The tumors tend to progress, and may extend along nerve roots to eventually involve the spinal roots and spinal cord. This process is almost always a manifestation of NEUROFIBROMATOSIS 1. (From Adams et al., Principles of Neurology, 6th ed, p1016; J Pediatr 1997 Nov;131(5):678-82)
INTERNEURONS of the vertebrate RETINA. They integrate, modulate, and interpose a temporal domain in the visual message presented to the RETINAL GANGLION CELLS, with which they synapse in the inner plexiform layer.
Neurons of the innermost layer of the retina, the internal plexiform layer. They are of variable sizes and shapes, and their axons project via the OPTIC NERVE to the brain. A small subset of these cells act as photoreceptors with projections to the SUPRACHIASMATIC NUCLEUS, the center for regulating CIRCADIAN RHYTHM.
A benign neoplasm that usually arises from the sympathetic trunk in the mediastinum. Histologic features include spindle cell proliferation (resembling a neurofibroma) and the presence of large ganglion cells. The tumor may present clinically with HORNER SYNDROME or diarrhea due to ectopic production of vasoactive intestinal peptide. (From DeVita et al., Cancer: Principles and Practice of Oncology, 5th ed, p966)
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