Malignant peripheral nerve sheath tumor classification: Difference between revisions
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==Overview== | ==Overview== | ||
A '''malignant peripheral nerve sheath tumor''' (MPNST) or '''malignant neurolemmoma''' is a form of [[cancer]] of the [[connective tissue]] surrounding [[nerve]]s. Given its origin and behavior it is classified as a [[sarcoma]]. About half the cases are diagonosed in people with [[neurofibromatosis]]; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8-13% (Evans ''et al'' 2002). The first-line treatmenet is surgical resection with wide margins. [[Chemotherapy]] (e.g. high-dose [[doxorubicin]]) and often [[radiation therapy|radiotherapy]] are done as adjuvant and/or neoadjuvant treatment. | ==Classification== | ||
A '''malignant peripheral nerve sheath tumor''' (MPNST) or '''malignant neurolemmoma''' is a form of [[cancer]] of the [[connective tissue]] surrounding [[nerve]]s. Given its origin and behavior it is classified as a [[sarcoma]]. About half the cases are diagonosed in people with [[neurofibromatosis]]; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8-13% (Evans ''et al'' 2002). The first-line treatmenet is surgical resection with wide margins. [[Chemotherapy]] (e.g. high-dose [[doxorubicin]]) and often [[radiation therapy|radiotherapy]] are done as adjuvant and/or neoadjuvant treatment.<ref name="pmid12011145">{{cite journal |author=Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A |title=Malignant peripheral nerve sheath tumours in neurofibromatosis 1 |journal=[[Journal of Medical Genetics]] |volume=39 |issue=5 |pages=311–4 |year=2002 |month=May |pmid=12011145 |pmc=1735122 |doi= |url=http://jmg.bmj.com/cgi/pmidlookup?view=long&pmid=12011145 |accessdate=2012-09-11}}</ref> | |||
==References== | ==References== |
Revision as of 14:09, 11 September 2012
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Classification
A malignant peripheral nerve sheath tumor (MPNST) or malignant neurolemmoma is a form of cancer of the connective tissue surrounding nerves. Given its origin and behavior it is classified as a sarcoma. About half the cases are diagonosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8-13% (Evans et al 2002). The first-line treatmenet is surgical resection with wide margins. Chemotherapy (e.g. high-dose doxorubicin) and often radiotherapy are done as adjuvant and/or neoadjuvant treatment.[1]
References
- ↑ Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A (2002). "Malignant peripheral nerve sheath tumours in neurofibromatosis 1". Journal of Medical Genetics. 39 (5): 311–4. PMC 1735122. PMID 12011145. Retrieved 2012-09-11. Unknown parameter
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