Difference between revisions of "Neurothekeoma"

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#redirect [[Peripheral_nerve_sheath_tumours#Neurothekeoma]]
'''Neurothekeoma''' is a benign [[peripheral nerve sheath tumours|peripheral nerve sheath tumour]].
 
It is also known as '''myxoma of the nerve sheath''' and '''nerve sheath myxoma'''.
 
There is growing evidence that neurothekomas and dermal nerve sheath myxomas are distinct entities.<ref>{{Cite journal  | last1 = Sheth | first1 = S. | last2 = Li | first2 = X. | last3 = Binder | first3 = S. | last4 = Dry | first4 = SM. | title = Differential gene expression profiles of neurothekeomas and nerve sheath myxomas by microarray analysis. | journal = Mod Pathol | volume = 24 | issue = 3 | pages = 343-54 | month = Mar | year = 2011 | doi = 10.1038/modpathol.2010.203 | PMID = 21297585 }}</ref>
 
==General==
*Rare.
*Female > male.
 
==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>
*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:<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>
*Cellular.
*[[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>
 
Images:
*[http://en.wikipedia.org/wiki/File:Neurothekeoma2.JPG Neurothekeoma (WP)].
*[http://path.upmc.edu/cases/case586.html Neurothekeoma - several images (upmc.edu)].
*[http://www.dermpedia.org/files/images/Image2_49.jpg Nerve sheath myxoma (dermpedia)]]
 
==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.
 
Exclusionary:
*S100 -ve.
**Exclude other peripheral nerve sheath tumours. (???)
**Myxoid variant +ve. {{fact}}
 
==See also==
*[[Peripheral nerve sheath tumours]].
 
==References==
{{Reflist|1}}


[[Category:Diagnosis]]
[[Category:Diagnosis]]

Revision as of 14:02, 7 February 2018

Neurothekeoma is a benign peripheral nerve sheath tumour.

It is also known as myxoma of the nerve sheath and nerve sheath myxoma.

There is growing evidence that neurothekomas and dermal nerve sheath myxomas are distinct entities.[1]

General

  • Rare.
  • Female > male.

Microscopic

Features:[2]

  • 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:[3]

  • Cellular.
  • Myxoid.
  • Intermediate.

DDx:

Images:

IHC

Features:[2]

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

Others:[5]

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

Exclusionary:

  • S100 -ve.
    • Exclude other peripheral nerve sheath tumours. (???)
    • Myxoid variant +ve. [citation needed]

See also

References

  1. ↑ Sheth, S.; Li, X.; Binder, S.; Dry, SM. (Mar 2011). "Differential gene expression profiles of neurothekeomas and nerve sheath myxomas by microarray analysis.". Mod Pathol 24 (3): 343-54. doi:10.1038/modpathol.2010.203. PMID 21297585.
  2. ↑ 2.0 2.1 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.
  3. ↑ 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.
  4. ↑ URL: http://surgpathcriteria.stanford.edu/softfib/angiomatoid_fibrous_histiocytoma/. Accessed on: 11 May 2011.
  5. ↑ 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.
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