Difference between revisions of "Peripheral nerve sheath tumours"

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Peripheral nerve sheath tumours (view source)

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==Malignant peripheral nerve sheath tumour==
==Malignant peripheral nerve sheath tumour==
*[[AKA]] ''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>
{{Main|Malignant peripheral nerve sheath tumour}}
*Commonly abbreviated ''MPNST''.
 
===General===
*Malignant - as the name implies.
*Usu. 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:
*[http://commons.wikimedia.org/wiki/File:Malignant_peripheral_nerve_sheath_tumour_-_intermed_mag.jpg MPNST - intermed. mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Malignant_peripheral_nerve_sheath_tumour_-_high_mag.jpg MPNST - high mag. (WC)].
*[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.


==Malignant triton tumour==
==Malignant triton tumour==
Michael
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