Malignant peripheral nerve sheath tumor pathophysiology: Difference between revisions
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==Overview== | ==Overview== | ||
== | ==Pathogenesis== | ||
Malignant peripheral nerve sheath tumors are a rare type of cancer that arise from the [[soft tissue]] that surrounds [[nerves]]. They are a type of [[sarcoma]]. Most malignant peripheral nerve sheath tumors arise from the [[nerve plexus]]es that distribute nerves into the limbs—the [[brachial plexus|brachial]] and [[lumbar plexus|lumbar]] plexuses—or from nerves as they arise from the trunk.<ref name="Panigrahi">{{Cite journal | pmid = 24174807| doi = 10.4103/0976-3147.116480| title = Primary malignant peripheral nerve sheath tumor at unusual location| journal = Journal of Neurosciences in Rural Practice| volume = 4| issue = 5| pages = 83| year = 2013| last1 = Panigrahi | first1 = S. | last2 = Mishra | first2 = S. | last3 = Das | first3 = S. | last4 = Dhir | first4 = M. | pmc=3808069}}</ref> | |||
==Genetics== | |||
About half of the cases of malignant peripheral nerve sheath tumor (MPNST) occur along with [[NF1]] | * 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 [[tumour suppressor]]; inactivation of the gene thus predisposes to [[tumour]] 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== | ||
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[[Category:Disease]] | [[Category:Disease]] | ||
[[Category:Types of cancer]] | [[Category:Types of cancer]] | ||
[[Category:Neurology]] | [[Category:Neurology]] | ||
Revision as of 14:35, 2 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
Pathogenesis
Malignant peripheral nerve sheath tumors are a rare type of cancer that arise from the soft tissue that surrounds nerves. They are a type of sarcoma. Most malignant peripheral nerve sheath tumors arise from the nerve plexuses that distribute nerves into the limbs—the brachial and lumbar plexuses—or from nerves as they arise from the trunk.[1]
Genetics
- 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.[2]
- The NF1 gene locus is on chromosome 17q11.2 and the gene product is neurofibromin, acts as a tumour suppressor; inactivation of the gene thus predisposes to tumour development.[3]
References
- ↑ Panigrahi, S.; Mishra, S.; Das, S.; Dhir, M. (2013). "Primary malignant peripheral nerve sheath tumor at unusual location". Journal of Neurosciences in Rural Practice. 4 (5): 83. doi:10.4103/0976-3147.116480. PMC 3808069. PMID 24174807.
- ↑ 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.