Difference between revisions of "Peripheral nerve sheath tumours"

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Peripheral nerve sheath tumours (view source)

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→‎Schwannoma: split-out
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=Specific diagnoses=
=Specific diagnoses=
==Schwannoma==  
==Schwannoma==  
===General===
{{Main|Schwannoma}}
*A common [[neuropathology]] [[CNS tumours|tumour]] that occasionally shows-up elsewhere.
*Tumour of tissue surrounding a nerve.
**Axons adjacent to the tumour are normal... but may be compressed.
*May be a part of [[neurofibromatosis type 2]].
 
===Microscopic===
Features:<ref name=pmid17893219>{{cite journal |author=Wippold FJ, Lubner M, Perrin RJ, Lämmle M, Perry A |title=Neuropathology for the neuroradiologist: Antoni A and Antoni B tissue patterns |journal=AJNR Am J Neuroradiol |volume=28 |issue=9 |pages=1633–8 |year=2007 |month=October |pmid=17893219 |doi=10.3174/ajnr.A0682 |url=http://www.ajnr.org/cgi/reprint/28/9/1633}}</ref>
*Antoni A:
**Cellular.
**'Fibrillary, polar, elongated'.
*Antoni B:
**Pauci-cellular.
**Loose microcystic tissue.
*Verocay bodies - paucinuclear area surrounded by nuclei - '''diagnostic feature'''.
*Hyaline thickened [[blood vessel]]s.
*Thick capsule.
*In the GI tract: classically have a ''peripheral lymphoid cuff''.<ref name=pmid15728600>{{cite journal |author=Levy AD, Quiles AM, Miettinen M, Sobin LH |title=Gastrointestinal schwannomas: CT features with clinicopathologic correlation |journal=AJR Am J Roentgenol |volume=184 |issue=3 |pages=797–802 |year=2005 |month=March |pmid=15728600 |doi= |url=http://www.ajronline.org/cgi/content/full/184/3/797}}</ref>
*+/-Hemosiderin deposition within tumour.
 
Notes:
*Tumour does ''not'' smear well.<ref>MUN. 24 November 2010.</ref>
*Antoni A: may look somewhat like scattered matchsticks.
 
DDx:
*[[Meningioma]].
*[[Intranodal palisaded myofibroblastoma]] - if surrounded by a rim of lymphoid tissue, i.e. [[Lymph node pathology|intranodal]].
*[[Leiomyoma]].
 
Images:
*www:
**[http://www.pathguy.com/~lulo/lulo0003.htm Antoni A (pathguy.com)].
**[http://www.ajnr.org/cgi/content/full/28/9/1633/F8 Antoni A & Antoni B side-by-side (ajnr.org)].
**[http://path.upmc.edu/cases/case639.html Cystic schwannoma - several images (upmc.edu)].
*[[WC]]:
**[http://commons.wikimedia.org/wiki/File:Schwannoma_-_Antoni_A_and_B_-_intermed_mag.jpg Schwannoma - Antoni A & B - intermed. mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Schwannoma_-_Antoni_A_and_B_-_very_high_mag.jpg Scwhannoma - Antoni A & B - very high mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Psammomatous_melanotic_schwannoma_-_high_mag.jpg Psammomatous melanotic schwannoma - high mag. (WC)].
**[http://commons.wikimedia.org/wiki/File:Nerve_root_schwannoma_-_intermed_mag.jpg Nerve root schwannoma - intermed. mag. (WC)].
 
====Schwannoma subtypes====
There are four:<ref name=pmid12792904>{{cite journal |author=Kurtkaya-Yapicier O, Scheithauer B, Woodruff JM |title=The pathobiologic spectrum of Schwannomas |journal=Histol. Histopathol. |volume=18 |issue=3 |pages=925–34 |year=2003 |month=July |pmid=12792904 |doi= |url=}}</ref>
#Conventional schwannoma.
#Cellular schwannoma.
#Plexiform schwannoma.
#Melanotic schwannoma.
 
=====Conventional schwannoma=====
*Most common.
 
=====Cellular schwannoma=====
*May mimic [[MPNST]].
 
Images:
*[http://path.upmc.edu/cases/case518.html Cellular schwannoma (upmc.edu)].
 
=====Plexiform schwannoma=====
*May mimic [[MPNST]] if cellular - esp. in childhood.
 
Images:
*[http://commons.wikimedia.org/wiki/File:Plexiform_Schwannoma_2.jpg Plexiform schwannoma - low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Plexiform_Schwannoma_1.jpg Plexiform schwannoma - high mag. (WC)].
 
=====Melanotic schwannoma=====
*May be confused with [[melanoma]].
*Psammomatous form (''psammomatous melanotic schwannoma'') associated with a heritable disorder ([[Carney complex]]).
 
Note:
*[[Carney complex]]:<ref name=pmid12792904/>
**Cutaneous lentigines.
**Myxomas (skin (subcutaneous), subcutanous, [[Atrial myxoma|heart]]).
**Endocrine neoplasms.
 
Images:
*[http://path.upmc.edu/cases/case387.html Psammomatous melanotic schwannoma - several images (upmc.edu)].
 
===IHC===
Features:<ref name=pmid12692193>{{cite journal |author=Hirose T, Tani T, Shimada T, Ishizawa K, Shimada S, Sano T |title=Immunohistochemical demonstration of EMA/Glut1-positive perineurial cells and CD34-positive fibroblastic cells in peripheral nerve sheath tumors |journal=Mod. Pathol. |volume=16 |issue=4 |pages=293–8 |year=2003 |month=April |pmid=12692193 |doi=10.1097/01.MP.0000062654.83617.B7 |url=http://www.nature.com/modpathol/journal/v16/n4/full/3880761a.html }}</ref>
*S-100 +ve.
*Glut1 +ve.
*CD34 +ve.
*Cytokeratins ~70% +ve.{{fact}}
*SOX10 +ve.<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>
**-ve in [[synovial sarcoma]], [[rhabdomyosarcoma]], [[chondrosarcoma]].
*EMA -ve. (???)
**Usually +ve (~75% of the time) in meningiomas.<ref>{{Cite journal  | last1 = Rushing | first1 = EJ. | last2 = Bouffard | first2 = JP. | last3 = McCall | first3 = S. | last4 = Olsen | first4 = C. | last5 = Mena | first5 = H. | last6 = Sandberg | first6 = GD. | last7 = Thompson | first7 = LD. | title = Primary extracranial meningiomas: an analysis of 146 cases. | journal = Head Neck Pathol | volume = 3 | issue = 2 | pages = 116-30 | month = Jun | year = 2009 | doi = 10.1007/s12105-009-0118-1 | PMID = 19644540 }}
</ref>


==Perineurioma==
==Perineurioma==
Michael
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