Advertisement

Topics

Study of Plexiform Neurofibromas in Neurofibromatosis Type 1

2014-08-27 03:57:18 | BioPortfolio

Summary

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.

Description

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.

Study Design

N/A

Conditions

Neurofibroma, Plexiform

Location

National Cancer Institute (NCI)
Bethesda
Maryland
United States
20892

Status

Active, not recruiting

Source

National Institutes of Health Clinical Center (CC)

Results (where available)

View Results

Links

Published on BioPortfolio: 2014-08-27T03:57:18-0400

Clinical Trials [22 Associated Clinical Trials listed on BioPortfolio]

Study of PEG-Intron for Plexiform Neurofibromas

This study is for slow growing tumors called plexiform neurofibromas (PNF) which are a relatively common problem in people with neurofibromatosis type 1 (NF1). These tumors are benign but...

Pirfenidone in Treating Young Patients With Neurofibromatosis Type 1 and Plexiform Neurofibromas

RATIONALE: Chemoprevention therapy is the use of certain drugs to try to prevent the development or recurrence of cancer. Pirfenidone may slow the growth or prevent further development of ...

Fludeoxyglucose F 18 Positron Emission Tomography and Magnetic Resonance Perfusion Imaging in Patients With Neurofibromatosis 1 and Plexiform Neurofibroma

RATIONALE: New imaging procedures such as fludeoxyglucose F 18 positron emission tomography (FDG-PET) and magnetic resonance (MR) perfusion imaging may improve the ability to detect diseas...

Childhood Cancer and Plexiform Neurofibroma Tissue Microarray for Molecular Target Screening and Clinical Drug Development

This study will construct tissue microarrays (TMAs) pertaining to childhood cancer. TMA technology is a recently developed one that allows for evaluating hundreds of tissue samples simulta...

Pilot Study of Gleevec/Imatinib Mesylate (STI-571, NSC 716051) in Neurofibromatosis (NF1) Patient With Plexiform Neurofibromas

This is a second Pilot Study to determine the efficacy of Gleevec® in neurofibromatosis (NF1) patients with plexiform neurofibromas using new response assessment modalities with the secon...

PubMed Articles [62 Associated PubMed Articles listed on BioPortfolio]

Long-Term Results for a One-Stage Surgery Technique for Patients With Craniofacial Plexiform Neurofibroma.

Neurofibromatosis (NF) is an autosomal dominant genetic disorder, and NF type 1 (NF1) is one of the most common forms. Plexiform neurofibroma (PNF) is one of the characteristic expressions of NF1. The...

Low-fat, plexiform spindle cell lipoma of the lip expressing S100 protein: a neural tumor simulator.

The plexiform variant of spindle cell lipoma is very uncommon. In fact, as far as we are aware only seven cases have been previously reported. We describe herein the case of a 49-year-old man with a s...

Neurofibromatous neuropathy: An ultrastructural study.

Plexiform neurofibroma is pathognomonic of neurofibromatosis 1 (NF1). An NF1-associated peripheral neuropathy has been described in a small minority of NF1 patients but its histopathological features ...

Transoral resection of extensive pediatric supraglottic neurofibroma.

This case report describes the novel use of transoral robotic surgery (TORS) with delayed transoral laser microsurgery (TLM) for residual disease in the resection of a pediatric neurofibroma with supr...

Prevalence of ALK gene alterations among the spectrum of plexiform spitzoid lesions.

ALK gene rearrangements have been described in spitzoid lesions with plexiform growth pattern.

Medical and Biotech [MESH] Definitions

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)

Neoplasms which arise from nerve sheaths formed by SCHWANN CELLS in the PERIPHERAL NERVOUS SYSTEM or by OLIGODENDROCYTES in the CENTRAL NERVOUS SYSTEM. Malignant peripheral nerve sheath tumors, NEUROFIBROMA, and NEURILEMMOMA are relatively common tumors in this category.

More From BioPortfolio on "Study of Plexiform Neurofibromas in Neurofibromatosis Type 1"

Advertisement
Quick Search
Advertisement
Advertisement

 

Relevant Topics

Pediatrics
Pediatrics is the general medicine of childhood. Because of the developmental processes (psychological and physical) of childhood, the involvement of parents, and the social management of conditions at home and at school, pediatrics is a specialty. With ...

Bioinformatics
Bioinformatics is the application of computer software and hardware to the management of biological data to create useful information. Computers are used to gather, store, analyze and integrate biological and genetic information which can then be applied...

Wound management
Anything that breaks the skin is a wound because when the skin is broken, there's a risk of germs getting into the body and causing an infection. Follow and track Wound Care News on BioPortfolio: Wound Car...


Searches Linking to this Trial