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clear cell sarcoma of soft tissue

Saturday 15 November 2003

soft parts melanoma, melanoma of soft parts, clear cell sarcoma of tendons and aponeuroses, clear cell sarcoma of soft tissues

Digital cases

- Case 206 (HPC:206) : Clear cell sarcoma of soft parts
- Case 218 (HPC:218) : Clear cell sarcoma of soft parts

Images

- Clear cell sarcoma of soft tissue

Definition: Clear cell sarcoma is now included under the uncertain differentiation heading (OMS, 2004) because, despite its clearly neuroectodermal nature.

Localization

- tendon and fascia
- bone
- digestive system (17000668)

Cytogenetics

- t(12;22)(q13;q12) causing a fusion of the EWSR1 and ATF1 genes

Cytogenetics in the differential diagnosis of clear cell sarcoma (melanoma of the soft parts) and primary cutaneous or metastatic melanoma

Clear cell sarcoma, in contrast to melanoma, has been found to contain a balanced t(12;22)(q13;q13) aberration in 50–75% of cases.

Molecular biology

- EWSR1/ATF1 fusion transcript by t(12;22)(q13;q12)
- EWSR1/CREB1 fusion transcript by a t(12;22) (17000668)

Expression profiling: 15150091

Differential diagnosis

-  sarcomas with clear cells
- sarcomas with epitheioid cells
- if giant cell : giant cell tumors

References

- Antonescu CR, Nafa K, Segal NH, Dal Cin P, Ladanyi M. EWS-CREB1: a recurrent variant fusion in clear cell sarcoma—association with gastrointestinal location and absence of melanocytic differentiation. Clin Cancer Res. 2006 Sep 15;12(18):5356-62. PMID: 17000668