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Difference between revisions of "Malignant peripheral nerve sheath tumour"
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Malignant peripheral nerve sheath tumour (view source)
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{{ Infobox diagnosis | |||
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| LMDDx = [[synovial sarcoma]], [[fibrosarcoma]], cellular [[schwannoma]], plexiform schwannoma, [[malignant triton tumour]]. | |||
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| Site = [[soft tissue lesions|soft tissue]] | |||
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'''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> and '''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> | |||
==General== | |||
*Malignant - as the name implies. | |||
*Usually assoc. with a peripheral nerve.{{Fact}} | |||
*May be seen in the context of [[neurofibromatosis type 1]]. | |||
==Microscopic== | |||
Features: | |||
*Cellular. | |||
*Nuclear atypia. | |||
*Mitoses. | |||
*+/-Herring bone pattern. | |||
Notes: | |||
*May be diagnosed in a poorly diff. tumour if patient has [[NF1]]. | |||
DDx: | |||
*Cellular [[schwannoma]]. | |||
*Plexiform schwannoma. | |||
*[[Malignant triton tumour]]. | |||
DDx of herring bone: | |||
*MPNST. | |||
*[[Synovial sarcoma]]. | |||
*[[Fibrosarcoma]]. | |||
===Images=== | |||
<gallery> | |||
Image:Malignant_peripheral_nerve_sheath_tumour_-_intermed_mag.jpg | MPNST - intermed. mag. (WC) | |||
Image:Malignant_peripheral_nerve_sheath_tumour_-_high_mag.jpg | MPNST - high mag. (WC) | |||
</gallery> | |||
www: | |||
*[http://path.upmc.edu/cases/case112/micro.html MPNST - several images (upmc.edu)]. | |||
*[http://path.upmc.edu/cases/case406.html MPNST - case 2 - several images (upmc.edu)]. | |||
===Grading=== | |||
*Can be graded histologically,<ref name=pmid14508395>{{cite journal |author=Zhou H, Coffin CM, Perkins SL, Tripp SR, Liew M, Viskochil DH |title=Malignant peripheral nerve sheath tumor: a comparison of grade, immunophenotype, and cell cycle/growth activation marker expression in sporadic and neurofibromatosis 1-related lesions |journal=Am. J. Surg. Pathol. |volume=27 |issue=10 |pages=1337–45 |year=2003 |month=October |pmid=14508395 |doi= |url=}}</ref> and this is prognostic.<ref name=pmid16923196>{{cite journal |author=Kar M, Deo SV, Shukla NK, ''et al.'' |title=Malignant peripheral nerve sheath tumors (MPNST)--clinicopathological study and treatment outcome of twenty-four cases |journal=World J Surg Oncol |volume=4 |issue= |pages=55 |year=2006 |pmid=16923196 |pmc=1560134 |doi=10.1186/1477-7819-4-55 |url=}}</ref> | |||
Sarcoma grading system<ref name=pmid6693192>{{cite journal |author=Trojani M, Contesso G, Coindre JM, ''et al.'' |title=Soft-tissue sarcomas of adults; study of pathological prognostic variables and definition of a histopathological grading system |journal=Int. J. Cancer |volume=33 |issue=1 |pages=37–42 |year=1984 |month=January |pmid=6693192 |doi= |url=}}</ref> - based on: | |||
*Tumour differentiation. | |||
*Mitotic rate. | |||
*[[Necrosis]]. | |||
==IHC== | |||
Features:<ref name=pmid18636017>{{cite journal |author=Nonaka D, Chiriboga L, Rubin BP |title=Sox10: a pan-schwannian and melanocytic marker |journal=Am. J. Surg. Pathol. |volume=32 |issue=9 |pages=1291–8 |year=2008 |month=September |pmid=18636017 |doi=10.1097/PAS.0b013e3181658c14 |url=}}</ref> | |||
*S-100 +ve ~ 30% of tumours. | |||
*SOX10 +ve ~ 50% of tumours. | |||
Others:<ref name=pmid14508395>{{cite journal |author=Zhou H, Coffin CM, Perkins SL, Tripp SR, Liew M, Viskochil DH |title=Malignant peripheral nerve sheath tumor: a comparison of grade, immunophenotype, and cell cycle/growth activation marker expression in sporadic and neurofibromatosis 1-related lesions |journal=Am. J. Surg. Pathol. |volume=27 |issue=10 |pages=1337–45 |year=2003 |month=October |pmid=14508395 |doi= |url=}}</ref> | |||
*p53. | |||
*p16. | |||
*p27. | |||
*MIB1. | |||
==See also== | |||
*[[Peripheral nerve sheath tumours]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||
[[Category:Peripheral nerve sheath tumours]] | |||