The diversity of soft tissue tumours with EWSR1 gene rearrangements: a review
Author:
- 6 November 2013
- journal article
- review article
- Published by Wiley in Histopathology
- Vol. 64 (1) , 134-150
Abstract
Many soft tissue sarcomas have chromosomal translocations with resultant formation of new fusion genes. Among the genes that can be rearranged, the EWSR1 gene has been identified as a partner in a wide variety of clinically and pathologically diverse sarcomas as well as some non‐mesenchymal tumours. The former include Ewing sarcoma and similar (Ewing‐like) small round cell sarcomas, desmoplastic small round cell tumour, myxoid liposarcoma, extraskeletal myxoid chondrosarcoma, angiomatoid fibrous histiocytoma, clear cell sarcoma of soft tissue and clear cell sarcoma‐like tumours of the gastrointestinal tract, primary pulmonary myxoid sarcoma, extrasalivary myoepithelial tumours and sporadic examples of low‐grade fibromyxoid sarcoma, sclerosing epithelioid fibrosarcoma and mesothelioma. EWSR1 is a ‘promiscuous’ gene that can fuse with many different partner genes, but sometimes this results in phenotypically identical tumours. EWSR1 can, conversely, partner with the same genes in morphologically and behaviourally different neoplasms. This paper reviews the diversity of the several soft tissue tumour types that are associated with rearrangement of the EWSR1 gene.Keywords
This publication has 193 references indexed in Scilit:
- Clear cell sarcoma expressing a novel chimerical transcript EWSR1 exon 7/ATF1 exon 6Virchows Archiv, 2012
- High prevalence of CIC fusion with double‐homeobox (DUX4) transcription factors in EWSR1‐negative undifferentiated small blue round cell sarcomasGenes, Chromosomes and Cancer, 2011
- Identification of a novel, recurrent HEY1‐NCOA2 fusion in mesenchymal chondrosarcoma based on a genome‐wide screen of exon‐level expression dataGenes, Chromosomes and Cancer, 2011
- Discovery of two novel EWSR1/ATF1 transcripts in four chimerical transcripts-expressing clear cell sarcoma and their quantitative evaluationExperimental and Molecular Pathology, 2011
- Clinical features and outcomes in patients with extraskeletal ewing sarcomaCancer, 2011
- EWSR1‐POU5F1 fusion in soft tissue myoepithelial tumors. A molecular analysis of sixty‐six cases, including soft tissue, bone, and visceral lesions, showing common involvement of the EWSR1 geneGenes, Chromosomes and Cancer, 2010
- EZH2 is a mediator of EWS/FLI1 driven tumor growth and metastasis blocking endothelial and neuro-ectodermal differentiationProceedings of the National Academy of Sciences, 2009
- Long-term survival and transmission of INI1-mutation via nonpenetrant males in a family with rhabdoid tumour predisposition syndromeBritish Journal of Cancer, 2007
- Ewing sarcoma gene EWS is essential for meiosis and B lymphocyte developmentJournal of Clinical Investigation, 2007
- The impact of translocations and gene fusions on cancer causationNature Reviews Cancer, 2007