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'''Peripheral nerve sheath tumours''', abbreviated '''PNSTs''', are common in [[neuropathology]] and occasionally show-up elsewhere.  A very common PNST is the [[Peripheral nerve sheath tumours#Schwannoma|schwannoma]].
'''Peripheral nerve sheath tumours''', abbreviated '''PNSTs''', are common in [[neuropathology]] and occasionally show-up elsewhere.  A very common PNST is the [[schwannoma]].


==Classification==
=Classification=
A classification:<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>
A classification:<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>
*Benign:
*Benign:
**Schwannoma.
**[[Schwannoma]].
**Neurofibroma.
**[[Neurofibroma]].
**Perineurioma.
**[[Perineurioma]].
**Traumatic neuroma.
**[[Traumatic neuroma]].
*Malignant:
*Malignant:
**Malignant peripheral nerve sheath tumour (MPNST).
**[[Malignant peripheral nerve sheath tumour]] (MPNST).


=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===
==Perineurioma==
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>
{{Main|Perineurioma}}
*Antoni A:
**Cellular.
**'Fibrillary, polar, elongated'.
*Antoni B:
**Pauci-cellular.
**Loose microcystic tissue.
*Verocay bodies - paucinuclear area surrounded by nuclei.
*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>


Notes:
==Traumatic neuroma==
*Tumour does ''not'' smear well.<ref>MUN. 24 November 2010.</ref>
:May be referred to as ''[[neuroma]]''.
*Antoni A: may look somewhat like scattered matchsticks.
{{Main|Traumatic neuroma}}
 
Micrographs:
*[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)].
 
====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.
#*Most common.
#Cellular.
#*May mimic [[MPNST]].
#Plexiform.
#*May mimic [[MPNST]] if cellular - esp. in childhood.
#Melanotic.
#*May be confused with [[melanoma]].
#*Psammomatous form associated with a heritable disorder (Carney complex).


Notes:
==Palisaded encapsulated neuroma==
*Carney complex:<ref name=pmid12792904/>
*Abbreviated ''PEN''.
**Cutaneous lentigines.
*[[AKA]] ''palisaded and encapsulated neuroma''.
**Myxomas (skin (subcutaneous), subcutanous, [[Cardiac tumours#Atrial myxoma|heart]]).
*[[AKA]] ''solitary circumscribed neuroma''.
**Endocrine neoplasms.


===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]].
==Perineurioma==
===General===
===General===
*Benign tumour derived from perineurial cells.
*Flesh-colour [[papule]] - classically on the face.<ref name=Ref_Derm536>{{Ref Derm|536}}</ref>
*Isolated finding - not associated with a systemic disease or malignancy.<ref name=pmid18718196>{{Cite journal  | last1 = Newman | first1 = MD. | last2 = Milgraum | first2 = S. | title = Palisaded encapsulated neuroma (PEN): an often misdiagnosed neural tumor. | journal = Dermatol Online J | volume = 14 | issue = 7 | pages = 12 | month =  | year = 2008 | doi =  | PMID = 18718196 }}</ref>
*Superficial skin papule.<ref>S. Sade. 8 September 2011.</ref>
*It is considered hyperplastic rather than neoplastic. <ref>Rosai & Ackermann, Surgical Pathology, 10th ed. p183</ref>


===Microscopic===
===Microscopic===
Features:<ref name=pc_neuri>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970452-1 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970452-1]. Accessed on: 13 May 2011.</ref>
Features:<ref name=Ref_Derm536>{{Ref Derm|536}}</ref>
*Long slender spindle cells with:
*Encapsulated dermal spindle cell lesion.
**Small spindle-shaped nuclei.
**Fasciular arrangement.
**Neural-type spindle cells:  
**#Not vacuolated.
**#Nuclei have pointy ends.
**#Sometimes epitheloid appearance.  
*Intralesional clefts.
**Useful to differentiate from schwannoma.


DDx:
DDx:
*Neuroma.
*[[Schwannoma]]:<ref name=Ref_Derm536>{{Ref Derm|536}}</ref>
*Neurofibroma.
**No intralesional clefts.
*Schwannoma.
**More variability in the cellularity.
**May be deep.
 
Other considerations:
*[[Leiomyoma]] - cytoplasm not vacuolated, nuclei more elliptical.  


Image:
Images:
*[http://www.pathconsultddx.com/pathCon/largeImage?pii=S1559-8675%2806%2970452-1&figureId=fig2 Perineurioma (pathconsultddx.com)].
*[http://dermatology.cdlib.org/147/case_presentation/pen/2.jpg PEN (cdlib.org)].<ref name=pmid18718196>{{Cite journal  | last1 = Newman | first1 = MD. | last2 = Milgraum | first2 = S. | title = Palisaded encapsulated neuroma (PEN): an often misdiagnosed neural tumor. | journal = Dermatol Online J | volume = 14 | issue = 7 | pages = 12 | month =  | year = 2008 | doi =  | PMID = 18718196 }}</ref>


===IHC===
<gallery>
Features:<ref name=pc_neuri>URL: [http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970452-1 http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675%2806%2970452-1]. Accessed on: 13 May 2011.</ref>
File:Palisaded_and_Encapsulated_Neuroma_(3952635881).jpg | Palisaded and encapsulated neuroma (Ed Uthman)
*S100 -ve.
File:Palisaded_and_Encapsulated_Neuroma,_S-100_Immunostain_(3953412396).jpg| PEN, S-100 staining (Ed Uthman)
*EMA +ve.


==Traumatic neuroma==
</gallery>
===General===
[[File:569 dp sl 1.png| Palisading/encapcuslated neuroma]]
*Consequence of trauma -- diagnosis requires history of trauma.
[[File:569 dp sl 2.png| Palisading/encapcuslated neuroma]]<br>
Palisading/encapsulated (Reed’s) neuroma. A. A dermal nodule shows an attenuated capsule (black arrows) about a fasciculated spindle cell lesion with artefactual clefts (green arrows). B. The bland spindled nuclei, amid clear cytoplasm with thin eosinophilic wisps, are often wavy (black arrows), with pointed ends (green arrows); leiomyomas have blunt ended nuclei and more eosinophilic cytoplasm.  These benign neoplasms, unlike neurofibromas, lack an association with neurofibromatosis.


===Microscopic===
===IHC===
Features:
Features:<ref name=pmid18718196/>
*Nerve with adjacent small organized micro-fascicles in collagen - as seen in regeneration.
*S100 +ve.
*EMA +ve (capsule of lesion).


==Neurofibroma==
==Neurofibroma==
===General===
{{Main|Neurofibroma}}
*May be a part of [[neurofibromatosis]] 1.
Includes discussion of ''plexiform neurofibroma''.
*Composed of Schwann cells, axons, fibrous material.<ref name=pmid17893219/>


===Microscopic===
==Neurothekeoma==
Features:<ref name=pmid17893219/>
{{Main|Neurothekeoma}}
*Plexiform growth pattern - "bag of worms".


DDx:
==Malignant peripheral nerve sheath tumour==
*[[Schwannoma]].
{{Main|Malignant peripheral nerve sheath tumour}}
*[[Dermatofibrosarcoma protuberans]] (DFSP) - S-100 -ve, CD34 +ve.
*[[Ganglioneuroma]].


Image:
==Malignant triton tumour==
*[http://commons.wikimedia.org/wiki/File:Neurofibroma_(1).jpg Neurofibroma - low mag. (WC)].
*Abbreviated ''MTT''.
*[http://commons.wikimedia.org/wiki/File:Neurofibroma_(2).jpg Neurofibroma - high mag. (WC)].
*[[AKA]] ''malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation''.<ref name=pmid17149968>{{Cite journal  | last1 = Stasik | first1 = CJ. | last2 = Tawfik | first2 = O. | title = Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation (malignant triton tumor). | journal = Arch Pathol Lab Med | volume = 130 | issue = 12 | pages = 1878-81 | month = Dec | year = 2006 | doi = 10.1043/1543-2165(2006)130[1878:MPNSTW]2.0.CO;2 | PMID = 17149968 }}</ref>
*[http://commons.wikimedia.org/wiki/File:Neurofibroma_(3).jpg Neurofibroma - high mag. (WC)].


===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>
*S100 +ve.
*CD34 +ve.
*Glut1 +ve.
*EMA +ve/-ve.
==Neurothekeoma==
===General===
===General===
*Rare.
*Rare.
*Female > male.
*Considered to be a variant of ''[[MPNST]]''.
*Prognosis worse that conventional MPNST.<ref name=pmid17149968/>
**Five year survival ~14%.<ref name=pmid22253011>{{Cite journal  | last1 = McConnell | first1 = YJ. | last2 = Giacomantonio | first2 = CA. | title = Malignant triton tumors-complete surgical resection and adjuvant radiotherapy associated with improved survival. | journal = J Surg Oncol | volume =  | issue =  | pages =  | month = Jan | year = 2012 | doi = 10.1002/jso.23042 | PMID = 22253011 }}</ref>
*Diagnosis may require clinical information, i.e. individual has a history of [[neurofibromatosis type 1]] (NF1).


Note:
*A handful of ''benign triton tumours'' are reported; these are considered ''neuromuscular [[hamartoma]]s''.<ref name=pmid15814954>{{Cite journal  | last1 = Castro | first1 = DE. | last2 = Raghuram | first2 = K. | last3 = Phillips | first3 = CD. | title = Benign triton tumor of the trigeminal nerve. | journal = AJNR Am J Neuroradiol | volume = 26 | issue = 4 | pages = 967-9 | month = Apr | year = 2005 | doi =  | PMID = 15814954 }}
</ref>
===Microscopic===
===Microscopic===
Features:<ref name=pmid17325474>{{cite journal |author=Hornick JL, Fletcher CD |title=Cellular neurothekeoma: detailed characterization in a series of 133 cases |journal=Am. J. Surg. Pathol. |volume=31 |issue=3 |pages=329–40 |year=2007 |month=March |pmid=17325474 |doi=10.1097/01.pas.0000213360.03133.89 |url=}}</ref>
Features - Woodruff criteria - all three required:<ref name=pmid17149968/>
*Dermal spindle cell lesion.
# (a) Tumour arise from a peripheral nerve ''or'' (b) individual has [[NF1]] ''or'' (c) lesion a metastasis arising in the context of (a) or (b).
*+/-Inflammation around lesion.
# Schwann cell tumour characteristics.
# Rhabdomyoblasts.
#* Eccentric nucleus.
#* Moderate amount of eosinophilic cytoplasm.
#* +/-Cross-striations.  


Notes:
DDx:
*No atypia.
*[[Malignant peripheral nerve sheath tumour]].
*Mitoses rare/none.
*[[Adult fibrosarcoma]].
 
*[[Synovial sarcoma]].
Subtypes:<ref name=pmid10555009>{{cite journal |author=Wang AR, May D, Bourne P, Scott G |title=PGP9.5: a marker for cellular neurothekeoma |journal=Am. J. Surg. Pathol. |volume=23 |issue=11 |pages=1401–7 |year=1999 |month=November |pmid=10555009 |doi= |url=}}</ref>
*[[Rhabdomyosarcoma]].
*Cellular.
*[[Carcinosarcoma]].
*Myxoid.
*Intermediate.
 
DDx:
*[[Dermatofibroma]].
*Angiomatoid fibrous histiocytoma -- have cystic blood filled spaces, inflammation.<ref>URL: [http://surgpathcriteria.stanford.edu/softfib/angiomatoid_fibrous_histiocytoma/ http://surgpathcriteria.stanford.edu/softfib/angiomatoid_fibrous_histiocytoma/]. Accessed on: 11 May 2011.</ref>
 
Image(s):
*[http://en.wikipedia.org/wiki/File:Neurothekeoma2.JPG Neurothekeoma (WP)].


===IHC===
===IHC===
Features:<ref name=pmid17325474/>
*NKI/C3 ([[AKA]] NKI-C3) +ve.
*NSE +/-ve.
Others:<ref name=pmid17592278>{{cite journal |author=Fetsch JF, Laskin WB, Hallman JR, Lupton GP, Miettinen M |title=Neurothekeoma: an analysis of 178 tumors with detailed immunohistochemical data and long-term patient follow-up information |journal=Am. J. Surg. Pathol. |volume=31 |issue=7 |pages=1103–14 |year=2007 |month=July |pmid=17592278 |doi=10.1097/PAS.0b013e31802d96af |url=}}</ref>
*Vimentin +ve.
*CD10 +ve.
*Microphthalmia transcription factor +ve.
*PGP9.5 +ve.
==Malignant peripheral nerve sheath tumours==
===General===
*Malignant - as the name implies.
*Usu. assoc. with a peripheral nerve.{{Fact}}
===Microscopic===
Features:
Features:
*Cellular.
*S100 +ve/-ve -- usu. focal if positive.<ref name=pmid17149968/>
*Nuclear atypia.
*Leu-7 +ve/-ve.
*Mitoses.
*Myelin basic protein +ve/-ve.
 
DDx:
*Cellular schwannoma.
*Plexiform schwannoma.


Image(s):
Rhabdomyoblastic differentiation:<ref name=pmid17149968/>
*[http://www.sarctrials.org/upload/mpnstpath_35923.jpg MPNST (sarctrials.org)].<ref>URL: [http://www.sarctrials.org/SARC006MPNST http://www.sarctrials.org/SARC006MPNST]. Accessed on: 5 December 2010.</ref>
*Desmin.
*Actin.
*Myogenin.


Notes:
===EM===
*May be diagnosed in a poorly diff. tumour if patient has NF1.
*+/-Sarcomeres.<ref name=pmid17149968/>
 
====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>
==Morton neuroma==
*p53.
:[[AKA]] ''plantar interdigital neuroma''.<ref name=pmid22995258>{{Cite journal | last1 = Makki | first1 = D. | last2 = Haddad | first2 = BZ. | last3 = Mahmood | first3 = Z. | last4 = Shahid | first4 = MS. | last5 = Pathak | first5 = S. | last6 = Garnham | first6 = I. | title = Efficacy of corticosteroid injection versus size of plantar interdigital neuroma. | journal = Foot Ankle Int | volume = 33 | issue = 9 | pages = 722-6 | month = Sep | year = 2012 | doi = DOI: 10.3113/FAI.2012.0722 | PMID = 22995258 }}</ref>
*p16.
{{Main|Morton neuroma}}
*p27.
*MIB-1.


==See also==
=See also=
*[[Neuropathology]].
*[[Neuropathology]].
*[[Gastrointestinal pathology]].
*[[Gastrointestinal pathology]].


==References==
=References=
{{Reflist|2}}
{{Reflist|2}}


[[Category:Neuropathology]]
[[Category:Neuropathology]]
[[Category:Peripheral nerve sheath tumours]]
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