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Difference between revisions of "Clear cell sarcoma"
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{{ Infobox diagnosis | |||
| Name = {{PAGENAME}} | |||
| Image = Clear_cell_sarcoma.Image12.jpg | |||
| Width = | |||
| Caption = Clear cell sarcoma. [[H&E stain]]. | |||
| Micro = tumour cells uniform - spindle-shaped ''or'' epithelioid, classically have clear cytoplasm and prominent nucleoli, +/-binucleation; sheeting ''or'' fascicular arrangement with fibrous septae - between tumour cells | |||
| Subtypes = | |||
| LMDDx = [[malignant melanoma]], [[PEComa]], carcinoma | |||
| Stains = | |||
| IHC = S100 +ve, HMB-45 +ve, Melan A (MART-1) usu. +ve; sometimes -ve, BCL2 +ve | |||
| EM = | |||
| Molecular = t(12;22) | |||
| IF = | |||
| Gross = | |||
| Grossing = | |||
| Site = [[soft tissue lesions|soft tissue]] | |||
| Assdx = | |||
| Syndromes = | |||
| Clinicalhx = | |||
| Signs = | |||
| Symptoms = | |||
| Prevalence = | |||
| Bloodwork = | |||
| Rads = | |||
| Endoscopy = | |||
| Prognosis = | |||
| Other = | |||
| ClinDDx = | |||
}} | |||
'''Clear cell sarcoma''', abbreviated '''CCS''', is an uncommon malignant soft tissue tumour. | |||
It is also known as '''soft-tissue melanoma''' and '''melanoma of the soft parts''', as it has a strong morphological resemblance to [[malignant melanoma]].<ref name=pmid18300804>{{cite journal |author=Hisaoka M, Ishida T, Kuo TT, ''et al.'' |title=Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases |journal=Am. J. Surg. Pathol. |volume=32 |issue=3 |pages=452–60 |year=2008 |month=March |pmid=18300804 |doi=10.1097/PAS.0b013e31814b18fb |url=}}</ref> | |||
It should '''not''' to be confused with ''[[clear cell sarcoma of the kidney]]''. | |||
==General== | |||
*Molecular changes and origin distinct from [[malignant melanoma]]. | |||
*Incidence: rare soft tissue tumour. | |||
===Clinical=== | |||
*Usually - deep soft tissue ''or'' extremities. | |||
**Classically associated with tendons and aponeuroses.<ref name=pmid17227118>{{Cite journal | last1 = Dim | first1 = DC. | last2 = Cooley | first2 = LD. | last3 = Miranda | first3 = RN. | title = Clear cell sarcoma of tendons and aponeuroses: a review. | journal = Arch Pathol Lab Med | volume = 131 | issue = 1 | pages = 152-6 | month = Jan | year = 2007 | doi = 10.1043/1543-2165(2007)131[152:CCSOTA]2.0.CO;2 | PMID = 17227118 }}</ref> | |||
*Guarded prognosis. | |||
*First described in 1965.<ref>URL: [http://www.informaworld.com/smpp/723576818-750600/ftinterface~db=all~content=a789166263~fulltext=713240928 http://www.informaworld.com/smpp/723576818-750600/ftinterface~db=all~content=a789166263~fulltext=713240928]. Accessed on: 5 May 2010.</ref> | |||
==Microscopic== | |||
Features:<ref name=pmid18300804/> | |||
*Architecture: sheeting ''or'' fascicular (bundles) arrangement. | |||
**Fibrous septae - between tumour cells. | |||
*Tumour cells uniform (low pleomorphism) - spindle-shaped ''or'' epithelioid: | |||
**Classically have clear cytoplasm. | |||
**Prominent nucleoli - basophilic - '''key feature'''. | |||
**+/-Binucleation. | |||
DDx: | |||
*[[Malignant melanoma]]. | |||
*[[PEComa]]. | |||
*Carcinoma. | |||
===Images=== | |||
<gallery> | |||
Image:Clear_cell_sarcoma_-_intermed_mag.jpg | Clear cell sarcoma - intermed. mag. (WC) | |||
Image:Clear_cell_sarcoma_-_very_high_mag.jpg | Clear cell sarcoma - very high mag. (WC) | |||
Image:Clear_cell_sarcoma.Image12.jpg | Clear cell sarcoma (WC/Gardner) | |||
</gallery> | |||
==IHC== | |||
Features:<ref name=pmid18300804/> | |||
*S100 +ve. | |||
*HMB-45 +ve. | |||
*Melan A (MART-1) +ve; sometimes -ve. | |||
*BCL2 +ve. | |||
*CD57 +ve (usually). | |||
Keratins: | |||
*EMA may be +ve. | |||
*CAM5.2 -ve. | |||
*AE1/AE3 -ve. | |||
==Molecular studies== | |||
*[[Chromosomal translocation]] t(12;22)(q13;q12).<ref name=pmid18300804/> | |||
**Fusion transcripts: | |||
***EWSR1-ATF1. | |||
****Same translocation in: ''[[myxoid liposarcoma]]'',<ref name=pmid21115923>{{Cite journal | last1 = Suzuki | first1 = K. | last2 = Matsui | first2 = Y. | last3 = Endo | first3 = K. | last4 = Kubo | first4 = T. | last5 = Hasegawa | first5 = T. | last6 = Kimura | first6 = T. | last7 = Ohtani | first7 = O. | last8 = Yasui | first8 = N. | title = Myxoid liposarcoma with EWS-CHOP type 1 fusion gene. | journal = Anticancer Res | volume = 30 | issue = 11 | pages = 4679-83 | month = Nov | year = 2010 | doi = | PMID = 21115923 }}</ref> and ''[[hyalinizing clear cell carcinoma]]''. | |||
***EWSR1-CREB1 (GI tract associated). | |||
==See also== | |||
*[[Soft tissue lesions]]. | |||
*[[Malignant melanoma]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Soft tissue lesions]] | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||