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Difference between revisions of "Malignant peripheral nerve sheath tumour"
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Malignant peripheral nerve sheath tumour (view source)
Revision as of 08:12, 9 May 2016
, 08:12, 9 May 2016terminology and missing citation added.
Jensflorian (talk | contribs) (Image, IHC update) |
Jensflorian (talk | contribs) (terminology and missing citation added.) |
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'''Malignant peripheral nerve sheath tumour''', abbreviated '''MPNST''', is an uncommon malignant tumour of the nerve sheath. | '''Malignant peripheral nerve sheath tumour''', abbreviated '''MPNST''', is an uncommon malignant tumour of the nerve sheath. | ||
It is also known '''neurofibrosarcoma'''<ref name=pmid21317712>{{Cite journal | last1 = Mills | first1 = AM. | last2 = Karamchandani | first2 = JR. | last3 = Vogel | first3 = H. | last4 = Longacre | first4 = TA. | title = Endocervical fibroblastic malignant peripheral nerve sheath tumor (neurofibrosarcoma): report of a novel entity possibly related to endocervical CD34 fibrocytes. | journal = Am J Surg Pathol | volume = 35 | issue = 3 | pages = 404-12 | month = Mar | year = 2011 | doi = 10.1097/PAS.0b013e318208f72e | PMID = 21317712 }}</ref> | It is also known '''neurofibrosarcoma'''<ref name=pmid21317712>{{Cite journal | last1 = Mills | first1 = AM. | last2 = Karamchandani | first2 = JR. | last3 = Vogel | first3 = H. | last4 = Longacre | first4 = TA. | title = Endocervical fibroblastic malignant peripheral nerve sheath tumor (neurofibrosarcoma): report of a novel entity possibly related to endocervical CD34 fibrocytes. | journal = Am J Surg Pathol | volume = 35 | issue = 3 | pages = 404-12 | month = Mar | year = 2011 | doi = 10.1097/PAS.0b013e318208f72e | PMID = 21317712 }}</ref>, '''neurogenic sarcoma'''.<ref name=pmid23139572>{{Cite journal | last1 = Sham | first1 = ME. | last2 = Ghorpade | first2 = A. | last3 = Shetty | first3 = S. | last4 = Hari | first4 = . | last5 = Vinay | first5 = . | title = Malignant peripheral nerve cell tumour. | journal = J Maxillofac Oral Surg | volume = 9 | issue = 1 | pages = 68-71 | month = Mar | year = 2010 | doi = 10.1007/s12663-010-0019-6 | PMID = 23139572 }}</ref> and '''malignant schwannoma''' - these terms are however depreceated.<ref>{WHOCNS2007}</ref> | ||
==General== | ==General== | ||
*Malignant - as the name implies. | *Malignant - as the name implies. | ||
*Usually assoc. with a peripheral nerve.{ | *Usually assoc. with a peripheral nerve.<ref>{WHOCNS2007}</ref> | ||
*May be seen in the context of [[neurofibromatosis type 1]]. | *May be seen in the context of [[neurofibromatosis type 1]]. | ||
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==Molecular== | ==Molecular== | ||
Features:<ref>{{Cite journal | last1 = Röhrich | first1 = M. | last2 = Koelsche | first2 = C. | last3 = Schrimpf | first3 = D. | last4 = Capper | first4 = D. | last5 = Sahm | first5 = F. | last6 = Kratz | first6 = A. | last7 = Reuss | first7 = J. | last8 = Hovestadt | first8 = V. | last9 = Jones | first9 = DT. | title = Methylation-based classification of benign and malignant peripheral nerve sheath tumors. | journal = Acta Neuropathol | volume = | issue = | pages = | month = Feb | year = 2016 | doi = 10.1007/s00401-016-1540-6 | PMID = 26857854 }}</ref> | Features:<ref>{{Cite journal | last1 = Röhrich | first1 = M. | last2 = Koelsche | first2 = C. | last3 = Schrimpf | first3 = D. | last4 = Capper | first4 = D. | last5 = Sahm | first5 = F. | last6 = Kratz | first6 = A. | last7 = Reuss | first7 = J. | last8 = Hovestadt | first8 = V. | last9 = Jones | first9 = DT. | title = Methylation-based classification of benign and malignant peripheral nerve sheath tumors. | journal = Acta Neuropathol | volume = | issue = | pages = | month = Feb | year = 2016 | doi = 10.1007/s00401-016-1540-6 | PMID = 26857854 }}</ref><ref>{{Cite journal | last1 = Hirbe | first1 = AC. | last2 = Dahiya | first2 = S. | last3 = Miller | first3 = CA. | last4 = Li | first4 = T. | last5 = Fulton | first5 = RS. | last6 = Zhang | first6 = X. | last7 = McDonald | first7 = S. | last8 = DeSchryver | first8 = K. | last9 = Duncavage | first9 = EJ. | title = Whole Exome Sequencing Reveals the Order of Genetic Changes during Malignant Transformation and Metastasis in a Single Patient with NF1-plexiform Neurofibroma. | journal = Clin Cancer Res | volume = 21 | issue = 18 | pages = 4201-11 | month = Sep | year = 2015 | doi = 10.1158/1078-0432.CCR-14-3049 | PMID = 25925892 }}</ref> | ||
* Atypical neurofibromas and low-grade MPNST have a common methylation profile and frequent losses of CDKN2A. | * Atypical neurofibromas and low-grade MPNST have a common methylation profile and frequent losses of CDKN2A. | ||
* A subset of MPNST show loss of trimethylation of histone H3 at lysine 27 (H3K27me3). | * A subset of MPNST show loss of trimethylation of histone H3 at lysine 27 (H3K27me3). | ||
* P53 and beta-spectrin mutations during progression reported. | |||
==See also== | ==See also== | ||
*[[Peripheral nerve sheath tumours]]. | *[[Peripheral nerve sheath tumours]]. | ||