Neurofibromatosis Type 2 (NF2) Natural History Consortium
Abstract
Neurofibromatosis 2 (NF2) is an autosomal dominant disorder characterized by the development of multiple nervous system tumors. All subjects develop bilateral vestibular schwanomas that lead to deafness and death if untreated. Subjects also tend to develop multiple meningiomas and spinal tumors, which result in significant motor and sensory deficits if left untreated. In the past decade, great strides have been made in terms of radiographic diagnosis, surgical approaches to these tumors, and understanding of the molecular biology of NF2. Unfortunately, similar advances in the understanding of the natural history of these tumors, fundamental to the evaluation of treatments, have not yet been made. The purpose of this study was to define the growth rates and clinical course of tumors associated with NF2. We accomplished this goal through the following steps: 1. Developed an international consortium of clinical centers with expertise in NF2, further expanding the infrastructure developed in the Natural History of Vestibular Schwannomas in NF2 US Army grant. All subjects were evaluated at local centers with full neurological, ophthalmological, radiographical, and audiometric evaluations and the data were sent to a centralized center for analysis. 2. Developed standardized volumetric analysis of intracranial and spinal tumors. 3. Formed an infrastructure for use in future clinical trials. All NF2 subjects identified at clinical centers will be categorized as potential subjects for future clinical trials. 4. Examined molecular and clinical features which may predict tumor behavior. This study led to a better understanding of the natural history and clinical course of tumors associated with NF2. An understanding of the natural history is also fundamental to the determination of efficacy of future medical or surgical therapies.
Document Details
- Document Type
- Technical Report
- Publication Date
- Jan 01, 2007
- Accession Number
- ADA484303
Entities
People
- William Slattery
Organizations
- House Ear Institute