EWSR1—The Most Common Rearranged Gene in Soft Tissue Lesions, Which Also Occurs in Different Bone Lesions: An Updated Review
| Autor: | Uta Flucke, Laura S. Hiemcke-Jiwa, Joost van Gorp, Vasiliki Siozopoulou, David Creytens, Bastiaan B J Tops, Max M. van Noesel |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
0301 basic medicine
Medicine (General) Clinical Biochemistry ANGIOMATOID FIBROUS HISTIOCYTOMA Review MOLECULAR ANALYSIS Biology Rare cancers Radboud Institute for Molecular Life Sciences [Radboudumc 9] medicine.disease_cause soft tissue tumors MALIGNANT-MELANOMA Fusion gene 03 medical and health sciences 0302 clinical medicine R5-920 Medicine and Health Sciences medicine FUSION GENE molecular TAF15 bone tumors Myoepithelial cell SCLEROSING EPITHELIOID FIBROSARCOMA medicine.disease Fusion protein GRADE FIBROMYXOID SARCOMA CHROMOSOME-TRANSLOCATION 030104 developmental biology EWSR1 030220 oncology & carcinogenesis Chromosomal region Cancer research pathology Human medicine Clear-cell sarcoma Sarcoma CLEAR-CELL-SARCOMA EXTRASKELETAL MYXOID CHONDROSARCOMA Carcinogenesis VASCULAR MALFORMATIONS |
| Zdroj: | Diagnostics Diagnostics, Vol 11, Iss 1093, p 1093 (2021) Diagnostics, 11, 6 DIAGNOSTICS Diagnostics, 11 |
| ISSN: | 2075-4418 |
| Popis: | Contains fulltext : 238889.pdf (Publisher’s version ) (Open Access) EWSR1 belongs to the FET family of RNA-binding proteins including also Fused in Sarcoma (FUS), and TATA-box binding protein Associated Factor 15 (TAF15). As consequence of the multifunctional role of EWSR1 leading to a high frequency of transcription of the chromosomal region where the gene is located, EWSR1 is exposed to aberrations such as rearrangements. Consecutive binding to other genes leads to chimeric proteins inducing oncogenesis. The other TET family members are homologous. With the advent of widely used modern molecular techniques during the last decades, it has become obvious that EWSR1 is involved in the development of diverse benign and malignant tumors with mesenchymal, neuroectodermal, and epithelial/myoepithelial features. As oncogenic transformation mediated by EWSR1-fusion proteins leads to such diverse tumor types, there must be a selection on the multipotent stem cell level. In this review, we will focus on the wide variety of soft tissue and bone entities, including benign and malignant lesions, harboring EWSR1 rearrangement. Fusion gene analysis is the diagnostic gold standard in most of these tumors. We present clinicopathologic, immunohistochemical, and molecular features and discuss differential diagnoses. |
| Databáze: | OpenAIRE |
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