Molecular, genetic, and cellular pathogenesis of neurofibromas and surgical implications

Update item information
Publication Type Journal Article
School or College School of Medicine
Department Neurosurgery
Creator Couldwell, William T.; Viskochil, David H.; Fults, Daniel Webster
Other Author Gottfried, Oren N.
Title Molecular, genetic, and cellular pathogenesis of neurofibromas and surgical implications
Date 2006
Description NEUROFIBROMATOSIS 1 (NF1) IS A common autosomal dominant disease characterized by complex and multicellular neurofibroma tumors. Significant advances have been made in the research of the cellular, genetic, and molecular biology of NF1. The NF1 gene was identified by positional cloning. The functions of its protein product, neurofibromin, in RAS signaling and in other signal transduction pathways are being elucidated, and the important roles of loss of heterozygosity and haploinsufficiency in tumorigenesis are better understood. The Schwann cell was discovered to be the cell of origin for neurofibromas, but understanding of a more complicated interplay of multiple cell types in tumorigenesis, specifically recruited heterogenous cell types such as mast cells and fibroblasts, has important implications for surgical therapy of these tumors. This review summarizes the most recent NF1 and neurofibroma literature describing the pathogenesis and treatment of nerve sheath tumors. Understanding the biological underpinnings of tumorigenesis in NF1 has implications for future surgical and medical management of neurofibromas.
Type Text
Publisher Wolters Kluwer (LWW)
Volume 58
Issue 1
First Page 1
Last Page 16
Subject Loss of heterozygosity; Malignant peripheral nerve sheath tumor; Plexiform Neurofibroma; Spinal neurofibroma
Subject LCSH Neurofibroma; Neurofibromatosis; Mice as laboratory animals
Language eng
Bibliographic Citation Gottfried, O. N., Viskochil, D. H., Fults, D. W., & Couldwell, W. T. (2006). Molecular, genetic, and cellular pathogenesis of neurofibromas and surgical implications. Neurosurgery, 58(1), 1-16.
Rights Management (c) Wolters Kluwer (LWW) http://lww.com
Format Medium application/pdf
Format Extent 977,947 bytes
Identifier ir-main,12728
ARK ark:/87278/s6v98sfr
Setname ir_uspace
Date Created 2012-06-13
Date Modified 2012-06-13
ID 704725
Reference URL https://collections.lib.utah.edu/ark:/87278/s6v98sfr
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