Difference between revisions of "Peripheral nerve sheath tumours"

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Peripheral nerve sheath tumours (view source)

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==Perineurioma==
==Perineurioma==
===General===
{{Main|Perineurioma}}
*Benign tumour derived from perineurial cells (intraneural or soft tissue).
**ICD-O 9571/0
** WHO grade I
*Rarely malignant soft tissue perineurioma.
 
Variant:
*Reticular perineurioma.<ref name=pmid11257623>{{Cite journal  | last1 = Graadt van Roggen | first1 = JF. | last2 = McMenamin | first2 = ME. | last3 = Belchis | first3 = DA. | last4 = Nielsen | first4 = GP. | last5 = Rosenberg | first5 = AE. | last6 = Fletcher | first6 = CD. | title = Reticular perineurioma: a distinctive variant of soft tissue perineurioma. | journal = Am J Surg Pathol | volume = 25 | issue = 4 | pages = 485-93 | month = Apr | year = 2001 | doi =  | PMID = 11257623 }}</ref>
 
===Macroscopy===
*Intraneural perineurioma: segmental tubular enlargement of the nerve.
*Soft tissue perineurioma: Well circumscribed, but not encapsulated.
 
===Microscopic===
Features:<ref name=Ref_Sternberg5_424>{{Ref Sternberg5|424}}</ref>
*Perineural epithelioid cells.
**Abundant pale, fluffy appearing cytoplasm.
 
Note:
*May be intraneural.<ref name=Ref_Sternberg5_424>{{Ref Sternberg5|424}}</ref>
**Typical pseudo-onion bulbs.
**Long considered hypertrophic neuropathy.
**Rare (less than 1% of all nerve sheath tumours).
 
DDx:
*[[Neuroma]].
*[[Neurofibroma]].
*[[Schwannoma]].
**S100 +ve, EMA -ve.<ref name=Ref_Sternberg5_424>{{Ref Sternberg5|424}}</ref>
*[[Liposarcoma]] - reticular perineurioma.
 
Images:
*[http://www.conganat.org/seap/reuniones/almagro2000/scruz/tnerviog5.htm Perineurioma (conganat.org)].
 
<gallery>
File:Perineurioma,_HE_stain.jpg | Pseudo-onion bulbs in a intraneural perioneurioma (WC/jensflorian)
</gallery>
 
===IHC===
Features:<ref name=pmid15958848>{{Cite journal  | last1 = Hornick | first1 = JL. | last2 = Fletcher | first2 = CD. | title = Soft tissue perineurioma: clinicopathologic analysis of 81 cases including those with atypical histologic features. | journal = Am J Surg Pathol | volume = 29 | issue = 7 | pages = 845-58 | month = Jul | year = 2005 | doi =  | PMID = 15958848 }}</ref><ref name=pmid1497116>{{Cite journal  | last1 = Tsang | first1 = WY. | last2 = Chan | first2 = JK. | last3 = Chow | first3 = LT. | last4 = Tse | first4 = CC. | title = Perineurioma: an uncommon soft tissue neoplasm distinct from localized hypertrophic neuropathy and neurofibroma. | journal = Am J Surg Pathol | volume = 16 | issue = 8 | pages = 756-63 | month = Aug | year = 1992 | doi =  | PMID = 1497116 }}</ref>
*S100 -ve.
*EMA +ve.
*CD34 ~65% +ve.<ref name=pmid15958848/>


==Traumatic neuroma==
==Traumatic neuroma==
===General===
:May be referred to as ''[[neuroma]]''.
*Consequence of trauma - diagnosis requires history of trauma.
{{Main|Traumatic neuroma}}
**May mimic a cancer recurrence at the site of a surgery.<ref name=pmid22330690/>
 
===Microscopic===
Features:<ref name=Ref_DCHH317>{{Ref DCHH|317}}</ref><ref name=pmid22330690/>
*+/-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:
*[[Morton neuroma]] (foot).
 
Images:
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3296625/figure/F2/ Traumatic neuroma (nih.gov)].<ref name=pmid22330690>{{Cite journal  | last1 = Li | first1 = Q. | last2 = Gao | first2 = EL. | last3 = Yang | first3 = YL. | last4 = Hu | first4 = HY. | last5 = Hu | first5 = XQ. | title = Traumatic neuroma in a patient with breast cancer after mastectomy: a case report and review of the literature. | journal = World J Surg Oncol | volume = 10 | issue =  | pages = 35 | month =  | year = 2012 | doi = 10.1186/1477-7819-10-35 | PMID = 22330690 }}</ref>
*[http://path.upmc.edu/cases/case502.html Traumatic neuroma - several images (upmc.edu)].
*[http://www.sarcomaimages.com/index.php?v=Neuroma,-Traumatic Traumatic neuroma (sarcomaimages.com)].
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2627419/figure/F1/ Traumatic neuroma (nih.gov)].<ref name=pmid17554193>{{Cite journal  | last1 = Kwon | first1 = JH. | last2 = Ryu | first2 = SW. | last3 = Kang | first3 = YN. | title = Traumatic neuroma around the celiac trunk after gastrectomy mimicking a nodal metastasis: a case report. | journal = Korean J Radiol | volume = 8 | issue = 3 | pages = 242-5 | month =  | year =  | doi =  | PMID = 17554193 | PMC = 2627419 }}</ref>
*[http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2835865/figure/F3/ Neuroma (nih.gov)].<ref name=pmid20224738>{{Cite journal  | last1 = Kitcat | first1 = M. | last2 = Hunter | first2 = JE. | last3 = Malata | first3 = CM. | title = Sciatic neuroma presenting forty years after above-knee amputation. | journal = Open Orthop J | volume = 3 | issue =  | pages = 125-7 | month =  | year = 2009 | doi = 10.2174/1874325000903010125 | PMID = 20224738 }}</ref>
 
===Sign out===
<pre>
SOFT TISSUE LESION, RIGHT WRIST, EXCISION:
- TRAUMATIC NEUROMA.
- BENIGN FIBROADIPOSE TISSUE.
</pre>
 
====Micro====
The sections show disordered nerve fibre bundles in fibrous tissue.


==Palisaded encapsulated neuroma==
==Palisaded encapsulated neuroma==
Line 145: Line 75:


==Neurothekeoma==
==Neurothekeoma==
*[[AKA]] ''myxoma of the nerve sheath'', [[AKA]] ''nerve sheath myxoma''.
{{Main|Neurothekeoma}}
*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}}


==Malignant peripheral nerve sheath tumour==
==Malignant peripheral nerve sheath tumour==
Michael
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