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==
*Abbreviated ''PEN''.
*Abbreviated ''PEN''.
*[[AKA]] ''palisaded and encapsulated neuroma''.
*[[AKA]] ''palisaded and encapsulated neuroma''.
*[[AKA]] ''solitary circumscribed neuroma''.
===General===
===General===
*Flesh-colour [[papule]] - classically on the face.<ref name=Ref_Derm536>{{Ref Derm|536}}</ref>
*Flesh-colour [[papule]] - classically on the face.<ref name=Ref_Derm536>{{Ref Derm|536}}</ref>
*Isolated finding - not associated with a systemic disease or malignancy.<ref name=pmid18718196>{{Cite journal  | last1 = Newman | first1 = MD. | last2 = Milgraum | first2 = S. | title = Palisaded encapsulated neuroma (PEN): an often misdiagnosed neural tumor. | journal = Dermatol Online J | volume = 14 | issue = 7 | pages = 12 | month =  | year = 2008 | doi =  | PMID = 18718196 }}</ref>
*Isolated finding - not associated with a systemic disease or malignancy.<ref name=pmid18718196>{{Cite journal  | last1 = Newman | first1 = MD. | last2 = Milgraum | first2 = S. | title = Palisaded encapsulated neuroma (PEN): an often misdiagnosed neural tumor. | journal = Dermatol Online J | volume = 14 | issue = 7 | pages = 12 | month =  | year = 2008 | doi =  | PMID = 18718196 }}</ref>
*Superficial skin.<ref>S. Sade. 8 September 2011.</ref>
*Superficial skin papule.<ref>S. Sade. 8 September 2011.</ref>
*It is considered hyperplastic rather than neoplastic. <ref>Rosai & Ackermann, Surgical Pathology, 10th ed. p183</ref>


===Microscopic===
===Microscopic===
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**#Not vacuolated.
**#Not vacuolated.
**#Nuclei have pointy ends.
**#Nuclei have pointy ends.
**#Sometimes epitheloid appearance.
*Intralesional clefts.
*Intralesional clefts.
**Useful to differentiate from schwannoma.
**Useful to differentiate from schwannoma.
Line 121: Line 55:
Images:
Images:
*[http://dermatology.cdlib.org/147/case_presentation/pen/2.jpg PEN (cdlib.org)].<ref name=pmid18718196>{{Cite journal  | last1 = Newman | first1 = MD. | last2 = Milgraum | first2 = S. | title = Palisaded encapsulated neuroma (PEN): an often misdiagnosed neural tumor. | journal = Dermatol Online J | volume = 14 | issue = 7 | pages = 12 | month =  | year = 2008 | doi =  | PMID = 18718196 }}</ref>
*[http://dermatology.cdlib.org/147/case_presentation/pen/2.jpg PEN (cdlib.org)].<ref name=pmid18718196>{{Cite journal  | last1 = Newman | first1 = MD. | last2 = Milgraum | first2 = S. | title = Palisaded encapsulated neuroma (PEN): an often misdiagnosed neural tumor. | journal = Dermatol Online J | volume = 14 | issue = 7 | pages = 12 | month =  | year = 2008 | doi =  | PMID = 18718196 }}</ref>
<gallery>
File:Palisaded_and_Encapsulated_Neuroma_(3952635881).jpg | Palisaded and encapsulated neuroma (Ed Uthman)
File:Palisaded_and_Encapsulated_Neuroma,_S-100_Immunostain_(3953412396).jpg| PEN, S-100 staining (Ed Uthman)
</gallery>
[[File:569 dp sl 1.png| Palisading/encapcuslated neuroma]]
[[File:569 dp sl 2.png| Palisading/encapcuslated neuroma]]<br>
Palisading/encapsulated (Reed’s) neuroma. A. A dermal nodule shows an attenuated capsule (black arrows) about a fasciculated spindle cell lesion with artefactual clefts (green arrows). B. The bland spindled nuclei, amid clear cytoplasm with thin eosinophilic wisps, are often wavy (black arrows), with pointed ends (green arrows); leiomyomas have blunt ended nuclei and more eosinophilic cytoplasm.  These benign neoplasms, unlike neurofibromas, lack an association with neurofibromatosis.


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