Clear Cell Sarcoma (CCS)
Melanoma of Soft Parts
Epidemiology and Etiology:
Common sites:
Gross features:
Histologic features:
Immunophenotype:
Marker:
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Sensitivity:
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Specificity:
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Molecular features:
Other features:
- t(12;22)(q13;q12) (~70% by karyotype with
histologic diagnosis of CCS)
- EWSR1/ATF1 fusion transcripts
- A few cases reported with other rearrangement of
either 12q13 or 22q12
- t(2;22)(q32;q12)
- EWSR1-CREB1
- GI tract preferentially
- Little or no melanocytic differentiation
- secondary abnormalities (almost all cases)
- +8 or i(8)(q10) (70%)
- +7
- -1p36
- +1q12-41
- -9p11
- +17q
- Hypodiploidy
to hypertriploidy
- No BRAF mutations
References:
- Sandberg AA & Bridge JA. Updates on the cytogenetics
and molecular genetics of bone and soft tissue tumors: clear cell sarcoma
(malignant melanoma of soft parts).
Cancer Genetics and Cytogenetics 130
(2001):1-7.
- Mitelman
& Heim. Cancer Cytogenetics (2009)