Ewing sarcoma and related FET family translocation-associated round cell tumors: A century of clinical and scientific progress Editorial


Authors: Maki, R. G.; Grohar, P. J.; Antonescu, C. R.
Title: Ewing sarcoma and related FET family translocation-associated round cell tumors: A century of clinical and scientific progress
Abstract: The year 2021 marked the centenary of the first publication of a cancer termed diffuse endothelioma of bone by James Ewing. Its unique features were apparent even in the first case series he described. This new diagnosis was clearly distinct from osteogenic sarcoma and myeloma, which were already well recognized at the time. We undertake this summary to better understanding Ewing sarcoma, contrasting the logarithmic evolution of the standard of care of systemic therapy for this and related diagnoses to the exponential understanding of the molecular biology of this family of tumors. We also outline in this manuscript how the finding of genomic relatives within Ewing sarcoma itself and related tumors, first noted nearly 40 years ago, helps us appreciate the need to find therapeutic plans that are specific for each small round blue cell tumor subtype. The advent of next generation sequencing regarding previously unknown small round blue cell tumor subtypes in many ways puts us back in the shoes of James Ewing in 1921, searching anew for clues leading to better treatments for increasingly rare cancer subsets. © 2022 Wiley Periodicals LLC.
Keywords: oncoprotein; gene translocation; genetics; systemic therapy; pathology; tumor marker; ewing sarcoma; sarcoma; oncogene proteins, fusion; translocation, genetic; translocation; sarcoma, ewing; humans; human; male; small round cell tumor; biomarkers, tumor; bcor::ccnb3; cic::dux4; fet sarcoma
Journal Title: Genes Chromosomes and Cancer
Volume: 61
Issue: 8
ISSN: 1045-2257
Publisher: Wiley Periodicals, Inc  
Date Published: 2022-08-01
Start Page: 509
End Page: 517
Language: English
DOI: 10.1002/gcc.23050
PUBMED: 35443099
PROVIDER: scopus
PMCID: PMC9197982
DOI/URL:
Notes: Article -- Export Date: 1 July 2022 -- Source: Scopus
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  1. Cristina R Antonescu
    895 Antonescu