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Difference between revisions of "Neurothekeoma"
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'''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]] | ||