Malignant peripheral nerve sheath tumor causes: Difference between revisions
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* About half of the cases of malignant peripheral nerve sheath tumor (MPNST) occur along with [[NF1]]. The lifetime risk of having both of these conditions is at 8–13% while those with only MPNST have a 0.001% in the general population.<ref>{{cite journal|last=Ferrari|first=Andrea|author2=Gianni Bisogno |author3=Modesto Carli |title=Management Of Childhood Malignant Peripheral Nerve Sheath Tumor | journal=Pediatric Drugs | date=2007|volume=9|issue=4|pages=239–48|accessdate=5 Mar 2014 | pmid=17705563 | doi=10.2165/00148581-200709040-00005}}</ref> | * About half of the cases of malignant peripheral nerve sheath tumor (MPNST) occur along with [[NF1]]. The lifetime risk of having both of these conditions is at 8–13% while those with only MPNST have a 0.001% in the general population.<ref>{{cite journal|last=Ferrari|first=Andrea|author2=Gianni Bisogno |author3=Modesto Carli |title=Management Of Childhood Malignant Peripheral Nerve Sheath Tumor | journal=Pediatric Drugs | date=2007|volume=9|issue=4|pages=239–48|accessdate=5 Mar 2014 | pmid=17705563 | doi=10.2165/00148581-200709040-00005}}</ref> | ||
* The [[NF1]] gene locus is on chromosome 17q11.2 and the gene product is [[neurofibromin]], acts as a tumor suppressor | * The [[NF1]] gene locus is on chromosome 17q11.2 and the gene product is [[neurofibromin]], which acts as a tumor suppressor. Inactivation of the gene predisposes to [[tumor]] development.<ref>{{cite book | last = Albright | first = A | title = Principles and practice of pediatric neurosurgery | publisher = Thieme | location = New York | year = 2008 | isbn = 1588903958 }}</ref> | ||
==References== | ==References== | ||
Revision as of 13:39, 15 December 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Shanshan Cen, M.D. [2]
Overview
Malignant peripheral nerve sheath tumor may be caused by a mutation in the neurofibromatosis type I gene.[1][2]
Causes
- Malignant peripheral nerve sheath tumor may be caused by a mutation in the neurofibromatosis type I gene.[3][4]
- About half of the cases of malignant peripheral nerve sheath tumor (MPNST) occur along with NF1. The lifetime risk of having both of these conditions is at 8–13% while those with only MPNST have a 0.001% in the general population.[5]
- The NF1 gene locus is on chromosome 17q11.2 and the gene product is neurofibromin, which acts as a tumor suppressor. Inactivation of the gene predisposes to tumor development.[6]
References
- ↑ Albright, A (2008). Principles and practice of pediatric neurosurgery. New York: Thieme. ISBN 1588903958.
- ↑ Ferrari, Andrea; Gianni Bisogno; Modesto Carli (2007). "Management Of Childhood Malignant Peripheral Nerve Sheath Tumor". Pediatric Drugs. 9 (4): 239–48. doi:10.2165/00148581-200709040-00005. PMID 17705563.
|access-date=requires|url=(help) - ↑ Albright, A (2008). Principles and practice of pediatric neurosurgery. New York: Thieme. ISBN 1588903958.
- ↑ Ferrari, Andrea; Gianni Bisogno; Modesto Carli (2007). "Management Of Childhood Malignant Peripheral Nerve Sheath Tumor". Pediatric Drugs. 9 (4): 239–48. doi:10.2165/00148581-200709040-00005. PMID 17705563.
|access-date=requires|url=(help) - ↑ Ferrari, Andrea; Gianni Bisogno; Modesto Carli (2007). "Management Of Childhood Malignant Peripheral Nerve Sheath Tumor". Pediatric Drugs. 9 (4): 239–48. doi:10.2165/00148581-200709040-00005. PMID 17705563.
|access-date=requires|url=(help) - ↑ Albright, A (2008). Principles and practice of pediatric neurosurgery. New York: Thieme. ISBN 1588903958.