Clear cell sarcoma
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Clear cell sarcoma, abbreviated CCS, is an uncommon malignant soft tissue tumour.
| 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) |
| Gross | deep location, usu. extremity |
| Site | soft tissue |
|
| |
| Signs | mass |
| Prevalence | uncommon |
| Prognosis | guarded |
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.
- Rare soft tissue tumour.[2]
- Guarded prognosis: 5 year and 10 year survival ~ 60% and 40% in one series.[2]
- First described in 1965.[3]
Gross
- Usually - deep soft tissue or extremities.
- Classically associated with tendons and aponeuroses.[4]
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:
- Malignant melanoma.
- PEComa.
- Carcinoma.
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
- Chromosomal translocation t(12;22)(q13;q12).[1]
- Fusion transcripts:
- EWSR1-ATF1.
- Same translocation in: myxoid liposarcoma,[5] and hyalinizing clear cell carcinoma.
- EWSR1-CREB1 (GI tract associated).
- EWSR1-ATF1.
- Fusion transcripts:
See also
References
- ↑ 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.
- ↑ 2.0 2.1 Hocar, O.; Le Cesne, A.; Berissi, S.; Terrier, P.; Bonvalot, S.; Vanel, D.; Auperin, A.; Le Pechoux, C. et al. (2012). "Clear cell sarcoma (malignant melanoma) of soft parts: a clinicopathologic study of 52 cases.". Dermatol Res Pract 2012: 984096. doi:10.1155/2012/984096. PMID 22693489.
- ↑ URL: http://www.informaworld.com/smpp/723576818-750600/ftinterface~db=all~content=a789166263~fulltext=713240928. Accessed on: 5 May 2010.
- ↑ 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.
- ↑ 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.