Difference between revisions of "Peripheral nerve sheath tumours"

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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==

Revision as of 13:57, 28 September 2013

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

General

  • Benign tumour derived from perineurial cells.

Variant:

  • Reticular perineurioma.[2]

Microscopic

Features:[3]

  • Perineural epithelioid cells.
    • Abundant pale, fluffy appearing cytoplasm.

Note:

  • May be intraneural.[3]

DDx:

Images:

IHC

Features:[4][5]

  • S100 -ve.
  • EMA +ve.
  • CD34 ~65% +ve.[4]

Traumatic neuroma

General

  • Consequence of trauma - diagnosis requires history of trauma.
    • May mimic a cancer recurrence at the site of a surgery.[6]

Microscopic

Features:[7][6]

  • +/-Nerve - that was injured.
  • Grouping of disordered nerve fibre bundles in fibrous tissue (collagen) - key feature.
  • +/-Myxoid change.
  • +/-Axonal swellings (ovoid pink/purple blobs).

DDx:

Images:

Sign out

SOFT TISSUE LESION, RIGHT WRIST, EXCISION:
- TRAUMATIC NEUROMA.
- BENIGN FIBROADIPOSE TISSUE.

Micro

The sections show disordered nerve fibre bundles in fibrous tissue.

Palisaded encapsulated neuroma

  • Abbreviated PEN.
  • AKA palisaded and encapsulated neuroma.

General

  • Flesh-colour papule - classically on the face.[9]
  • Isolated finding - not associated with a systemic disease or malignancy.[10]
  • Superficial skin.[11]

Microscopic

Features:[9]

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

DDx:

  • Schwannoma:[9]
    • No intralesional clefts.
    • More variability in the cellularity.
    • May be deep.

Other considerations:

  • Leiomyoma - cytoplasm not vacuolated, nuclei more elliptical.

Images:

IHC

Features:[10]

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

Neurofibroma

This section includes plexiform neurofibroma.

General

Classification:[12]

  1. Localized - sporatic.
  2. Diffuse - usually poorly defined, young adults and children; sporatic.
  3. Plexiform - associated with NF1.

Gross/radiologic

Gross features (plexiform NF):[12]

  • "Bag of worms" appearance.

Radiologic:[12]

  • Fusiform mass.

Microscopic

Features:

  • Spindle cells with wavy nuclei without pleomorphism - key feature.
    • Often described as "shredded carrots".
  • May be arranged in fascicles and intermixed with collagen.
    • Often no pattern is apparent.
  • Moderate increase of cellularity vis-a-vis normal dermis.
  • May be poorly or well-circumscribed.
  • +/-Plexiform growth pattern - "bag of worms".[1]
    • Multiple well-circumscribed nests.
  • Mast cells[13] - one has to look for them at high power.
    • Very useful for confirming the low power suspicion.

DDx:

Images

  • Neurofibroma - low mag. (WC)

  • Neurofibroma - intermed. mag. (WC)

  • Neurofibroma - high mag. (WC)

www:

IHC

Features:[16]

  • S100 +ve -- wavy pattern.[17]
  • CD34 +ve.
  • Glut1 +ve.
  • EMA +ve/-ve.
  • NF +ve/-ve.[17]
  • MART-1 -ve.[17]
    • Positive in neurotized melanocytic nevi.

Sign out

FOURTH TOE, LEFT, EXCISION:
- NEUROFIBROMA.

Micro

The sections show skin with a lesion composed of irregular-shaped groups of bland dermal spindle cells with wavy nuclei and pale-eosinophilic cytoplasm. Mast cells are seen scattered throughout the lesion. Thick collagen separates the clusters of the spindle cells. There is no nuclear atypia. Mitotic activity is not appreciated. No melanocytic nests are identified.

The overlying epidermis matures to the surface.

Alternate

The sections show skin with an unencapsulated dermal spindle cell lesion with navy nuclei that have a likeness to shredded carrots. Occasional mast cells are present within the lesion. There is no nuclear atypia. Mitotic activity is not appreciated. No melanocytic nests are identified.

Neurothekeoma

  • AKA myxoma of the nerve sheath, AKA nerve sheath myxoma.

General

  • Rare.
  • Female > male.

Microscopic

Features:[18]

  • Superficial dermal lesion:
    • Usu. lobulated or micronodular architecture - key feature.
      • +/-Focal sheeting.
    • Spindle/epithelioid morphology with pale eosinophilic cytoplasm - key feature.
    • +/-Inflammation around lesion.
    • +/-Surrounded by collagen.

Notes:

  • No atypia.
  • Mitoses rare/none.
  • Often poorly circumscribed.

Subtypes:[19]

  • Cellular.
  • Myxoid.
  • Intermediate.

DDx:

Images:

IHC

Features:[18]

  • NKI/C3 (AKA NKI-C3) +ve.
  • NSE +/-ve.

Others:[21]

  • Vimentin +ve.
  • CD10 +ve.
  • Microphthalmia transcription factor +ve.
  • PGP9.5 +ve.

Exclusionary:

  • S100 -ve.
    • Exclude other peripheral nerve sheath tumours. (???)

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.[22]

General

  • Rare.
  • Considered to be a variant of MPNST.
  • Prognosis worse that conventional MPNST.[22]
    • Five year survival ~14%.[23]
  • 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.[24]

Microscopic

Features - Woodruff criteria - all three required:[22]

  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.[22]
  • Leu-7 +ve/-ve.
  • Myelin basic protein +ve/-ve.

Rhabdomyoblastic differentiation:[22]

  • Desmin.
  • Actin.
  • Myogenin.

EM

Morton neuroma

General

  • Benign foot condition.
  • Uncommon.
  • Usu. interdigital nerves.

Etiology:

  • Indirect nerve trauma.

Clinical:[25]

  • Foot pain.

Microscopic

Features:[25]

  • Extensive fibrosis around and within the nerve.
  • Digital artery:

DDx:

Images:

See also

References

  1. ↑ 1.0 1.1 1.2 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. ↑ Graadt van Roggen, JF.; McMenamin, ME.; Belchis, DA.; Nielsen, GP.; Rosenberg, AE.; Fletcher, CD. (Apr 2001). "Reticular perineurioma: a distinctive variant of soft tissue perineurioma.". Am J Surg Pathol 25 (4): 485-93. PMID 11257623.
  3. ↑ 3.0 3.1 3.2 Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Reuter, Victor E; Stoler, Mark H (2009). Sternberg's Diagnostic Surgical Pathology (5th ed.). Lippincott Williams & Wilkins. pp. 424. ISBN 978-0781779425.
  4. ↑ 4.0 4.1 Hornick, JL.; Fletcher, CD. (Jul 2005). "Soft tissue perineurioma: clinicopathologic analysis of 81 cases including those with atypical histologic features.". Am J Surg Pathol 29 (7): 845-58. PMID 15958848.
  5. ↑ Tsang, WY.; Chan, JK.; Chow, LT.; Tse, CC. (Aug 1992). "Perineurioma: an uncommon soft tissue neoplasm distinct from localized hypertrophic neuropathy and neurofibroma.". Am J Surg Pathol 16 (8): 756-63. PMID 1497116.
  6. ↑ 6.0 6.1 6.2 Li, Q.; Gao, EL.; Yang, YL.; Hu, HY.; Hu, XQ. (2012). "Traumatic neuroma in a patient with breast cancer after mastectomy: a case report and review of the literature.". World J Surg Oncol 10: 35. doi:10.1186/1477-7819-10-35. PMID 22330690.
  7. ↑ Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 317. ISBN 978-0470519035.
  8. ↑ Kwon, JH.; Ryu, SW.; Kang, YN.. "Traumatic neuroma around the celiac trunk after gastrectomy mimicking a nodal metastasis: a case report.". Korean J Radiol 8 (3): 242-5. PMC 2627419. PMID 17554193. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2627419/.
  9. ↑ 9.0 9.1 9.2 Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 536. ISBN 978-0443066542.
  10. ↑ 10.0 10.1 10.2 Newman, MD.; Milgraum, S. (2008). "Palisaded encapsulated neuroma (PEN): an often misdiagnosed neural tumor.". Dermatol Online J 14 (7): 12. PMID 18718196.
  11. ↑ S. Sade. 8 September 2011.
  12. ↑ 12.0 12.1 12.2 Wilkinson, LM.; Manson, D.; Smith, CR. (Oct 2004). "Best cases from the AFIP: plexiform neurofibroma of the bladder.". Radiographics 24 Suppl 1: S237-42. doi:10.1148/rg.24si035170. PMID 15486243.
  13. ↑ Staser, K.; Yang, FC.; Clapp, DW. (Jul 2010). "Mast cells and the neurofibroma microenvironment.". Blood 116 (2): 157-64. doi:10.1182/blood-2009-09-242875. PMID 20233971.
  14. ↑ Gray, MH.; Smoller, BR.; McNutt, NS.; Hsu, A. (Jun 1990). "Neurofibromas and neurotized melanocytic nevi are immunohistochemically distinct neoplasms.". Am J Dermatopathol 12 (3): 234-41. PMID 1693815.
  15. ↑ Carr, NJ.; Warren, AY. (Jan 1993). "Mast cell numbers in melanocytic naevi and cutaneous neurofibromas.". J Clin Pathol 46 (1): 86-7. PMID 8432898.
  16. ↑ Hirose T, Tani T, Shimada T, Ishizawa K, Shimada S, Sano T (April 2003). "Immunohistochemical demonstration of EMA/Glut1-positive perineurial cells and CD34-positive fibroblastic cells in peripheral nerve sheath tumors". Mod. Pathol. 16 (4): 293–8. doi:10.1097/01.MP.0000062654.83617.B7. PMID 12692193. http://www.nature.com/modpathol/journal/v16/n4/full/3880761a.html.
  17. ↑ 17.0 17.1 17.2 Chen, Y.; Klonowski, PW.; Lind, AC.; Lu, D. (Jul 2012). "Differentiating neurotized melanocytic nevi from neurofibromas using Melan-A (MART-1) immunohistochemical stain.". Arch Pathol Lab Med 136 (7): 810-5. doi:10.5858/arpa.2011-0335-OA. PMID 22742554.
  18. ↑ 18.0 18.1 Hornick JL, Fletcher CD (March 2007). "Cellular neurothekeoma: detailed characterization in a series of 133 cases". Am. J. Surg. Pathol. 31 (3): 329–40. doi:10.1097/01.pas.0000213360.03133.89. PMID 17325474.
  19. ↑ Wang AR, May D, Bourne P, Scott G (November 1999). "PGP9.5: a marker for cellular neurothekeoma". Am. J. Surg. Pathol. 23 (11): 1401–7. PMID 10555009.
  20. ↑ URL: http://surgpathcriteria.stanford.edu/softfib/angiomatoid_fibrous_histiocytoma/. Accessed on: 11 May 2011.
  21. ↑ Fetsch JF, Laskin WB, Hallman JR, Lupton GP, Miettinen M (July 2007). "Neurothekeoma: an analysis of 178 tumors with detailed immunohistochemical data and long-term patient follow-up information". Am. J. Surg. Pathol. 31 (7): 1103–14. doi:10.1097/PAS.0b013e31802d96af. PMID 17592278.
  22. ↑ 22.0 22.1 22.2 22.3 22.4 22.5 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.
  23. ↑ 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.
  24. ↑ 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.
  25. ↑ 25.0 25.1 Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Oberman, Harold A; Reuter, Victor E (2004). Sternberg's Diagnostic Surgical Pathology (4th ed.). Lippincott Williams & Wilkins. pp. 243. ISBN 978-0781740517.
  26. ↑ URL: http://www.sciencedirect.com/science/article/pii/S1027811711000863. Accessed on: 27 December 2012.
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