Clear cell sarcoma

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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

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.
  • 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:

Images

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. 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.
  3. URL: http://www.informaworld.com/smpp/723576818-750600/ftinterface~db=all~content=a789166263~fulltext=713240928. Accessed on: 5 May 2010.
  4. 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.
  5. 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.