| Entry |
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| Name |
Clear cell sarcoma of soft tissue |
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| Supergrp |
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| Description |
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.
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| Category |
Cancer
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| Brite |
Human diseases in ICD-11 classification [BR:br08403]
02 Neoplasms
Malignant neoplasms, except primary neoplasms of lymphoid, haematopoietic, central nervous system or related tissues
Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
Malignant mesenchymal neoplasms
2B5K Unspecified malignant soft tissue tumours or sarcomas of bone or articular cartilage of other or unspecified sites
H00052 Clear cell sarcoma of soft tissue
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| Gene |
EWSR1-ATF1 (translocation) [HSA: 466] [KO: K09053]
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| Other DBs |
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| Reference |
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| Authors |
Malchau SS, Hayden J, Hornicek F, Mankin HJ |
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| Title |
Clear cell sarcoma of soft tissues. |
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| Journal |
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| Reference |
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| Authors |
Teixeira MR, Ribeiro FR, Cerveira N, Torres L, Amaro T, Henrique R, Lopes C |
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| Title |
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. |
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| Journal |
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| Reference |
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| Authors |
Letson GD, Muro-Cacho CA. |
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| Title |
Genetic and molecular abnormalities in tumors of the bone and soft tissues. |
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| Journal |
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| Reference |
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| Authors |
Helman LJ, Meltzer P. |
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| Title |
Mechanisms of sarcoma development. |
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| Journal |
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| Reference |
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| Authors |
Fisher C. |
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| Title |
Current aspects of the pathology of soft tissue sarcomas. |
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| Journal |
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| Reference |
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| Authors |
Kim YC, Vandersteen DP, Jung HG. |
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| Title |
Myxoid clear cell sarcoma. |
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| Journal |
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| Reference |
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| Authors |
Tong TR, Chow TC, Chan OW, Lee KC, Yeung SH, Lam A, Yu CK. |
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| Title |
Clear-cell sarcoma diagnosis by fine-needle aspiration: cytologic, histologic, and ultrastructural features; potential pitfalls; and literature review. |
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| Journal |
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| Reference |
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| Authors |
Creager AJ, Pitman MB, Geisinger KR. |
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| Title |
Cytologic features of clear cell sarcoma (malignant melanoma) of soft parts: a study of fine-needle aspirates and exfoliative specimens. |
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| Journal |
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