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 '''nerve sheath myxoma'''<ref name=pmid11070116>{{Cite journal  | last1 = Laskin | first1 = WB. | last2 = Fetsch | first2 = JF. | last3 = Miettinen | first3 = M. | title = The "neurothekeoma": immunohistochemical analysis distinguishes the true nerve sheath myxoma from its mimics. | journal = Hum Pathol | volume = 31 | issue = 10 | pages = 1230-41 | month = Oct | year = 2000 | doi = 10.1053/hupa.2000.18474 | PMID = 11070116 }}</ref> and '''myxoma of the nerve sheath'''.
 
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===
<gallery>
File:Neurothekeoma2.JPG | Neurothekeoma (WP)
</gallery>
====www====
*[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]] (MITF) +ve.
*PGP9.5 +ve.
*S100 +ve.<ref name=RefDerm541>{{Ref Derm|541}}</ref>
**-ve in older literature.
 
==See also==
*[[Peripheral nerve sheath tumours]].
 
==References==
{{Reflist|1}}
 
[[Category:Diagnosis]]

Latest revision as of 15:29, 5 November 2021

Neurothekeoma is a benign peripheral nerve sheath tumour.

It is also known as nerve sheath myxoma[1] and myxoma of the nerve sheath.

There is growing evidence that neurothekomas and dermal nerve sheath myxomas are distinct entities.[2]

General

  • Rare.
  • Female > male.

Microscopic

Features:[3]

  • 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:[4]

  • Cellular.
  • Myxoid.
  • Intermediate.

DDx:

Images

www

IHC

Features:[3]

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

Others:[6]

See also

References

  1. Laskin, WB.; Fetsch, JF.; Miettinen, M. (Oct 2000). "The "neurothekeoma": immunohistochemical analysis distinguishes the true nerve sheath myxoma from its mimics.". Hum Pathol 31 (10): 1230-41. doi:10.1053/hupa.2000.18474. PMID 11070116.
  2. 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.
  3. 3.0 3.1 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.
  4. 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.
  5. URL: http://surgpathcriteria.stanford.edu/softfib/angiomatoid_fibrous_histiocytoma/. Accessed on: 11 May 2011.
  6. 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.
  7. Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 541. ISBN 978-0443066542.