Difference between revisions of "Peripheral nerve sheath tumours"

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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]] [[CNS tumours|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>
*May be a part of [[neurofibromatosis type 2]].
*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 A:
*Encapsulated dermal spindle cell lesion.
**Cellular.
**Fasciular arrangement.
**'Fibrillary, polar, elongated'.
**Neural-type spindle cells:
*Antoni B:
**#Not vacuolated.
**Pauci-cellular.
**#Nuclei have pointy ends.
**Loose microcystic tissue.
**#Sometimes epitheloid appearance.  
*Verocay bodies - paucinuclear area surrounded by nuclei.
*Intralesional clefts.
*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>
**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.


Notes:
Other considerations:
*Tumour does ''not'' smear well.<ref>MUN. 24 November 2010.</ref>
*[[Leiomyoma]] - cytoplasm not vacuolated, nuclei more elliptical.  
*Antoni A: may look somewhat like scattered matchsticks.


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


====Subtypes====
<gallery>
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>
File:Palisaded_and_Encapsulated_Neuroma_(3952635881).jpg | Palisaded and encapsulated neuroma (Ed Uthman)
#Conventional.
File:Palisaded_and_Encapsulated_Neuroma,_S-100_Immunostain_(3953412396).jpg| PEN, S-100 staining (Ed Uthman)
#*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:
</gallery>
*Carney complex:<ref name=pmid12792904/>
[[File:569 dp sl 1.png| Palisading/encapcuslated neuroma]]
**Cutaneous lentigines.
[[File:569 dp sl 2.png| Palisading/encapcuslated neuroma]]<br>
**Myxomas (skin (subcutaneous), subcutanous, [[Cardiac tumours#Atrial myxoma|heart]]).
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.
**Endocrine neoplasms.


===IHC===
===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>
Features:<ref name=pmid18718196/>
*S-100 +ve.
*S100 +ve.
*Glut1 +ve.
*EMA +ve (capsule of lesion).
*CD34 +ve.
 
*Cytokeratins ~70% +ve.{{fact}}
==Neurofibroma==
*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>
{{Main|Neurofibroma}}
**-ve in synovial sarcoma, rhabomyosarcoma, chondrosarcoma.
Includes discussion of ''plexiform neurofibroma''.


==Traumatic neuroma==
==Neurothekeoma==
===General===
{{Main|Neurothekeoma}}
*Consequence of trauma -- diagnosis requires history of trauma.
 
==Malignant peripheral nerve sheath tumour==
{{Main|Malignant peripheral nerve sheath tumour}}


===Microscopic===
==Malignant triton tumour==
Features:
*Abbreviated ''MTT''.
*Nerve with adjacent small organized micro-fascicles in collagen - as seen in regeneration.
*[[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>


==Neurofibroma==
===General===
===General===
*May be a part of [[neurofibromatosis]] 1.
*Rare.
*Composed of Schwann cells, axons, fibrous material.<ref name=pmid17893219/>
*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=pmid17893219/>
Features - Woodruff criteria - all three required:<ref name=pmid17149968/>
*Plexiform growth pattern - "bag of worms".
# (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:
DDx:
*Schwannoma.
*[[Malignant peripheral nerve sheath tumour]].
*Dermatofibrosarcoma protuberans (DFSP) - S-100 -ve, CD34 +ve.
*[[Adult fibrosarcoma]].
*[[Ganglioneuroma]].
*[[Synovial sarcoma]].
 
*[[Rhabdomyosarcoma]].
Image:
*[[Carcinosarcoma]].
*[http://commons.wikimedia.org/wiki/File:Neurofibroma_(1).jpg Neurofibroma - low mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Neurofibroma_(2).jpg Neurofibroma - high mag. (WC)].
*[http://commons.wikimedia.org/wiki/File:Neurofibroma_(3).jpg Neurofibroma - high mag. (WC)].


===IHC===
===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.
==Malignant peripheral nerve sheath tumours==
===General===
*Malignant - as the name implies.
===Microscopic===
Features:
Features:
*Cellular.
*S100 +ve/-ve -- usu. focal if positive.<ref name=pmid17149968/>
*Mitoses.
*Leu-7 +ve/-ve.
*Myelin basic protein +ve/-ve.


DDx:
Rhabdomyoblastic differentiation:<ref name=pmid17149968/>
*Cellular schwannoma.
*Desmin.
*Plexiform schwannoma.
*Actin.
*Myogenin.


Image(s):
===EM===
*[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>
*+/-Sarcomeres.<ref name=pmid17149968/>


===IHC===
==Morton neuroma==
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>  
:[[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>
*S-100 +ve ~ 30% of tumours.
{{Main|Morton neuroma}}
*SOX10 +ve ~ 50% of tumours.


==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]]

Latest revision as of 14:18, 29 August 2018

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

Classification

A classification:[1]

Specific diagnoses

Schwannoma

Main article: Schwannoma

Perineurioma

Main article: Perineurioma

Traumatic neuroma

May be referred to as neuroma.
Main article: Traumatic neuroma

Palisaded encapsulated neuroma

  • Abbreviated PEN.
  • AKA palisaded and encapsulated neuroma.
  • AKA solitary circumscribed neuroma.

General

  • Flesh-colour papule - classically on the face.[2]
  • Isolated finding - not associated with a systemic disease or malignancy.[3]
  • Superficial skin papule.[4]
  • It is considered hyperplastic rather than neoplastic. [5]

Microscopic

Features:[2]

  • Encapsulated dermal spindle cell lesion.
    • Fasciular arrangement.
    • Neural-type spindle cells:
      1. Not vacuolated.
      2. Nuclei have pointy ends.
      3. Sometimes epitheloid appearance.
  • Intralesional clefts.
    • Useful to differentiate from schwannoma.

DDx:

  • Schwannoma:[2]
    • No intralesional clefts.
    • More variability in the cellularity.
    • May be deep.

Other considerations:

  • Leiomyoma - cytoplasm not vacuolated, nuclei more elliptical.

Images:

  • Palisaded and encapsulated neuroma (Ed Uthman)

  • PEN, S-100 staining (Ed Uthman)

Palisading/encapcuslated neuroma Palisading/encapcuslated neuroma
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:[3]

  • S100 +ve.
  • EMA +ve (capsule of lesion).

Neurofibroma

Main article: Neurofibroma

Includes discussion of plexiform neurofibroma.

Neurothekeoma

Main article: Neurothekeoma

Malignant peripheral nerve sheath tumour

Main article: Malignant peripheral nerve sheath tumour

Malignant triton tumour

  • Abbreviated MTT.
  • AKA malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation.[6]

General

  • Rare.
  • Considered to be a variant of MPNST.
  • Prognosis worse that conventional MPNST.[6]
    • Five year survival ~14%.[7]
  • 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 hamartomas.[8]

Microscopic

Features - Woodruff criteria - all three required:[6]

  1. (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).
  2. Schwann cell tumour characteristics.
  3. Rhabdomyoblasts.
    • Eccentric nucleus.
    • Moderate amount of eosinophilic cytoplasm.
    • +/-Cross-striations.

DDx:

IHC

Features:

  • S100 +ve/-ve -- usu. focal if positive.[6]
  • Leu-7 +ve/-ve.
  • Myelin basic protein +ve/-ve.

Rhabdomyoblastic differentiation:[6]

  • Desmin.
  • Actin.
  • Myogenin.

EM

  • +/-Sarcomeres.[6]

Morton neuroma

AKA plantar interdigital neuroma.[9]
Main article: Morton neuroma

See also

References

  1. ↑ Wippold FJ, Lubner M, Perrin RJ, Lämmle M, Perry A (October 2007). "Neuropathology for the neuroradiologist: Antoni A and Antoni B tissue patterns". AJNR Am J Neuroradiol 28 (9): 1633–8. doi:10.3174/ajnr.A0682. PMID 17893219. http://www.ajnr.org/cgi/reprint/28/9/1633.
  2. ↑ 2.0 2.1 2.2 Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 536. ISBN 978-0443066542.
  3. ↑ 3.0 3.1 3.2 Newman, MD.; Milgraum, S. (2008). "Palisaded encapsulated neuroma (PEN): an often misdiagnosed neural tumor.". Dermatol Online J 14 (7): 12. PMID 18718196.
  4. ↑ S. Sade. 8 September 2011.
  5. ↑ Rosai & Ackermann, Surgical Pathology, 10th ed. p183
  6. ↑ 6.0 6.1 6.2 6.3 6.4 6.5 Stasik, CJ.; Tawfik, O. (Dec 2006). "Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation (malignant triton tumor).". Arch Pathol Lab Med 130 (12): 1878-81. doi:10.1043/1543-2165(2006)130[1878:MPNSTW]2.0.CO;2. PMID 17149968.
  7. ↑ McConnell, YJ.; Giacomantonio, CA. (Jan 2012). "Malignant triton tumors-complete surgical resection and adjuvant radiotherapy associated with improved survival.". J Surg Oncol. doi:10.1002/jso.23042. PMID 22253011.
  8. ↑ Castro, DE.; Raghuram, K.; Phillips, CD. (Apr 2005). "Benign triton tumor of the trigeminal nerve.". AJNR Am J Neuroradiol 26 (4): 967-9. PMID 15814954.
  9. ↑ Makki, D.; Haddad, BZ.; Mahmood, Z.; Shahid, MS.; Pathak, S.; Garnham, I. (Sep 2012). "Efficacy of corticosteroid injection versus size of plantar interdigital neuroma.". Foot Ankle Int 33 (9): 722-6. doi:DOI: 10.3113/FAI.2012.0722. PMID 22995258.
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