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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==  
{{Main|Schwannoma}}
==Perineurioma==
{{Main|Perineurioma}}
==Traumatic neuroma==
:May be referred to as ''[[neuroma]]''.
{{Main|Traumatic neuroma}}
==Palisaded encapsulated neuroma==
*Abbreviated ''PEN''.
*[[AKA]] ''palisaded and encapsulated neuroma''.
*[[AKA]] ''solitary circumscribed neuroma''.
===General===
===General===
*A common [[neuropathology]] tumour that occasionally shows-up elsewhere.
*Flesh-colour [[papule]] - classically on the face.<ref name=Ref_Derm536>{{Ref Derm|536}}</ref>
*Tumour of tissue surrounding a nerve.
*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>
**Axons adjacent to the tumour are normal... but may be compressed.
*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=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>
Features:<ref name=Ref_Derm536>{{Ref Derm|536}}</ref>
*Antoni tissue (type A and type B).
*Encapsulated dermal spindle cell lesion.
*Verocay bodies - paucinuclear area surrounded by nuclei.
**Fasciular arrangement.
**Neural-type spindle cells:
**#Not vacuolated.
**#Nuclei have pointy ends.
**#Sometimes epitheloid appearance.
*Intralesional clefts.
**Useful to differentiate from schwannoma.
 
DDx:
*[[Schwannoma]]:<ref name=Ref_Derm536>{{Ref Derm|536}}</ref>
**No intralesional clefts.
**More variability in the cellularity.
**May be deep.
 
Other considerations:
*[[Leiomyoma]] - cytoplasm not vacuolated, nuclei more elliptical.
 
Images:
*[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>
 
<gallery>
File:Palisaded_and_Encapsulated_Neuroma_(3952635881).jpg | Palisaded and encapsulated neuroma (Ed Uthman)
File:Palisaded_and_Encapsulated_Neuroma,_S-100_Immunostain_(3953412396).jpg| PEN, S-100 staining (Ed Uthman)
 
</gallery>
[[File:569 dp sl 1.png| Palisading/encapcuslated neuroma]]
[[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.
 
===IHC===
Features:<ref name=pmid18718196/>
*S100 +ve.
*EMA +ve (capsule of lesion).
 
==Neurofibroma==
{{Main|Neurofibroma}}
Includes discussion of ''plexiform neurofibroma''.
 
==Neurothekeoma==
{{Main|Neurothekeoma}}
 
==Malignant peripheral nerve sheath tumour==
{{Main|Malignant peripheral nerve sheath tumour}}
 
==Malignant triton tumour==
*Abbreviated ''MTT''.
*[[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>
 
===General===
*Rare.
*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===
Features - Woodruff criteria - all three required:<ref name=pmid17149968/>
# (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).
# Schwann cell tumour characteristics.
# Rhabdomyoblasts.
#* Eccentric nucleus.
#* Moderate amount of eosinophilic cytoplasm.
#* +/-Cross-striations.
 
DDx:
*[[Malignant peripheral nerve sheath tumour]].
*[[Adult fibrosarcoma]].
*[[Synovial sarcoma]].
*[[Rhabdomyosarcoma]].
*[[Carcinosarcoma]].


Notes:
===IHC===
*Tumour does ''not'' smear well.<ref>MUN. 24 November 2010.</ref>
Features:
*S100 +ve/-ve -- usu. focal if positive.<ref name=pmid17149968/>
*Leu-7 +ve/-ve.
*Myelin basic protein +ve/-ve.


====Antoni A====
Rhabdomyoblastic differentiation:<ref name=pmid17149968/>
*Cellular.
*Desmin.
*'Fibrillary, polar, elongated'.
*Actin.
*Myogenin.


Comment: May look somewhat like scattered matchsticks.
===EM===
====Antoni B====
*+/-Sarcomeres.<ref name=pmid17149968/>
*Loose microcystic tissue.
*Adjacent to Antoni A.


Micrographs:
==Morton neuroma==
*[http://www.pathguy.com/~lulo/lulo0003.htm Antoni A (pathguy.com)].
:[[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>
*[http://www.ajnr.org/cgi/content/full/28/9/1633/F8 Antoni A & Antoni B side-by-side (ajnr.org)].
{{Main|Morton neuroma}}


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


==References==
=References=
{{Reflist|1}}
{{Reflist|2}}


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