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01-02-2010 | Review and Perspective

Soft tissue tumors associated with EWSR1 translocation

Authors: Salvatore Romeo, Angelo P. Dei Tos

Published in: Virchows Archiv | Issue 2/2010

Abstract

The Ewing sarcoma breakpoint region 1 (EWSR1; also known as EWS) represents one of the most commonly involved genes in sarcoma translocations. In fact, it is involved in a broad variety of mesenchymal lesions which includes Ewing's sarcoma/peripheral neuroectodermal tumor, desmoplastic small round cell tumor, clear cell sarcoma, angiomatoid fibrous histiocytoma, extraskeletal myxoid chondrosarcoma, and a subset of myxoid liposarcoma. The fusion products between EWSR1 and partners usually results in fusion of the N-terminal transcription-activating domain of EWSR1 and the C-terminal DNA-binding domain of the fusion partner, eventually generating novel transcription factors. EWSR1 rearrangement can be visualized by the means of fluorescence in situ hybridization (FISH). As soft tissue sarcomas represent a diagnostically challenging group, FISH analysis is an extremely useful confirmatory diagnostic tool. However, as in most instances a split-apart approach is used, the results of molecular genetics must be evaluated in context with morphology.

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Title: Soft tissue tumors associated with EWSR1 translocation
Authors: Salvatore Romeo
Angelo P. Dei Tos
Publication date: 01-02-2010
Publisher: Springer-Verlag
Published in: Virchows Archiv / Issue 2/2010
Print ISSN: 0945-6317
Electronic ISSN: 1432-2307
DOI: https://doi.org/10.1007/s00428-009-0854-3

Keynote webinar | Spotlight on medication adherence

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Soft tissue tumors associated with EWSR1 translocation

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Keynote webinar | Spotlight on medication adherence

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