Clear cell sarcoma of soft tissue

Definition:

Soft tissue sarcomas account for only about 1% of all malignant diseases and there are less than 8,700 new cases per year in the United States. Clear cell sarcoma (CCS), a highly malignant tumor of deep soft tissues is perhaps the rarest of these. CCS is characterized by the translocation t(12;22)(q13;q12). This translocation rearranges the ATF1 gene at 12q13 and the EWSR1 gene at 22q12, originating an EWSR1/ATF1 fusion gene. Given its specificity, this genetic aberration is considered pathognomonic for CCS and can be used as a diagnostic marker of this neoplasia.

References:

[1]. Andrew J Creager, et al. Cytologic features of clear cell sarcoma (malignant melanoma) of soft parts: a study of fine-needle aspirates and exfoliative specimens. Am J Clin Pathol. 2002 Feb;117(2):217-24. [Content Brief]

[2]. G D Letson, et al. Genetic and molecular abnormalities in tumors of the bone and soft tissues. Cancer Control. 2001 May-Jun;8(3):239-51. [Content Brief]

[3]. Lee J Helman, et al. Mechanisms of sarcoma development. Nat Rev Cancer. 2003 Sep;3(9):685-94. [Content Brief]

[4]. Manuel R Teixeira, et al. Karyotypic divergence and convergence in two synchronous lung metastases of a clear cell sarcoma of tendons and aponeuroses with t(12;22)(q13;q12) and type 1 EWS/ATF1. Cancer Genet Cytogenet. 2003 Sep;145(2):121-5. [Content Brief]

[5]. Sara Sofia Malchau, et al. Clear cell sarcoma of soft tissues. J Surg Oncol. 2007 May 1;95(6):519-22. [Content Brief]

[6]. Tommy R Tong, et al. Clear-cell sarcoma diagnosis by fine-needle aspiration: cytologic, histologic, and ultrastructural features; potential pitfalls; and literature review. Diagn Cytopathol. 2002 Mar;26(3):174-80. [Content Brief]

[7]. You Chan Kim, et al. Myxoid clear cell sarcoma. Am J Dermatopathol. 2005 Feb;27(1):51-5. [Content Brief]

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