Malignant peripheral nerve sheath tumor overview: Difference between revisions
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==Overview== | ==Overview== | ||
A '''malignant peripheral nerve sheath tumor''' (MPNST) 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]]. | A '''malignant peripheral nerve sheath tumor''' (MPNST) 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]]. | ||
A '''malignant peripheral nerve sheath tumor''' ('''MPNST''') (also known as "malignant schwannoma,"<ref name="Bolognia"/> "neurofibrosarcoma,"<ref name="Bolognia"/> and "neurosarcoma"<ref name="Bolognia">{{cite book |author=Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. |title=Dermatology: 2-Volume Set |publisher=Mosby |location=St. Louis |year=2007 |pages= |isbn=1-4160-2999-0 |oclc= |doi= |accessdate=}}</ref>) 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 diagnosed in people with [[neurofibromatosis]]; the lifetime risk for an MPNST in patients with [[neurofibromatosis type 1]] is 8-13%.<ref>{{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=J. Med. Genet. |volume=39 |issue=5 |pages=311–4 |date=May 2002 |pmid=12011145 |pmc=1735122 |doi= 10.1136/jmg.39.5.311|url=http://jmg.bmj.com/cgi/content/full/39/5/311}}</ref> MPNST with [[rhabdomyoblast]]omatous component are called [[malignant triton tumor]]s. | |||
The first-line treatment 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. | |||
==References== | ==References== | ||
Revision as of 16:21, 1 December 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
A malignant peripheral nerve sheath tumor (MPNST) 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.
A malignant peripheral nerve sheath tumor (MPNST) (also known as "malignant schwannoma,"[1] "neurofibrosarcoma,"[1] and "neurosarcoma"[1]) 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 diagnosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8-13%.[2] MPNST with rhabdomyoblastomatous component are called malignant triton tumors.
The first-line treatment is surgical resection with wide margins. Chemotherapy (e.g. high-dose doxorubicin) and often radiotherapy are done as adjuvant and/or neoadjuvant treatment.
References
- ↑ 1.0 1.1 1.2 Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN 1-4160-2999-0.
- ↑ Evans DG, Baser ME, McGaughran J, Sharif S, Howard E, Moran A (May 2002). "Malignant peripheral nerve sheath tumours in neurofibromatosis 1". J. Med. Genet. 39 (5): 311–4. doi:10.1136/jmg.39.5.311. PMC 1735122. PMID 12011145.