Difference between revisions of "Clear cell sarcoma"

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#redirect [[Soft_tissue_lesions#Clear_cell_sarcoma]]
{{ 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]]

Revision as of 05:14, 23 September 2013

Clear cell sarcoma
Diagnosis in short

Clear cell sarcoma. H&E stain.
LM 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
LM DDx malignant melanoma, PEComa, carcinoma
IHC S100 +ve, HMB-45 +ve, Melan A (MART-1) usu. +ve; sometimes -ve, BCL2 +ve
Molecular t(12;22)
Site soft tissue

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.[1]

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.[2]
  • Guarded prognosis.
  • First described in 1965.[3]

Microscopic

Features:[1]

  • 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:

Images

  • Clear cell sarcoma - intermed. mag. (WC)

  • Clear cell sarcoma - very high mag. (WC)

  • Clear cell sarcoma (WC/Gardner)

IHC

Features:[1]

  • 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

See also

References

  1. ↑ 1.0 1.1 1.2 1.3 Hisaoka M, Ishida T, Kuo TT, et al. (March 2008). "Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases". Am. J. Surg. Pathol. 32 (3): 452–60. doi:10.1097/PAS.0b013e31814b18fb. PMID 18300804.
  2. ↑ Dim, DC.; Cooley, LD.; Miranda, RN. (Jan 2007). "Clear cell sarcoma of tendons and aponeuroses: a review.". Arch Pathol Lab Med 131 (1): 152-6. doi:10.1043/1543-2165(2007)131[152:CCSOTA]2.0.CO;2. PMID 17227118.
  3. ↑ URL: http://www.informaworld.com/smpp/723576818-750600/ftinterface~db=all~content=a789166263~fulltext=713240928. Accessed on: 5 May 2010.
  4. ↑ Suzuki, K.; Matsui, Y.; Endo, K.; Kubo, T.; Hasegawa, T.; Kimura, T.; Ohtani, O.; Yasui, N. (Nov 2010). "Myxoid liposarcoma with EWS-CHOP type 1 fusion gene.". Anticancer Res 30 (11): 4679-83. PMID 21115923.
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