Ewing sarcomas and Ewing-like sarcomas : New aspects

Der Pathologe
K Specht, W Hartmann

Abstract

Sarcomas of the Ewing family of tumors are aggressive neoplasms occurring in bone and soft tissue of mostly children and young adults. Classical Ewing sarcomas are pathognomonically characterized by fusions between a gene of the RNA-binding TET family (EWSR1 or FUS) with a gene of the ETS-transcription family (FLI1, ERG, ETV1, ETV4 or FEV). Less frequent cases designated as Ewing-like sarcomas show different genetic rearrangements between EWSR1 and non-ETS genes (NFATC2, POU5F1, SMARCA5, PATZ, ZSG, SP3). Moreover, new molecular alterations biologically unrelated to Ewing sarcomas have recently been described in the category of undifferentiated round cell sarcomas including CIC-DUX4 fusions or BCOR alterations, each carrying unique gene expression signatures. In contrast to classical Ewing sarcomas, the morphologic spectrum of these tumor entities is much broader and includes round cell areas as well as spindled and myxoid components. The immunohistochemical profile with inconsistent CD99 positivity makes diagnosis more difficult and requires the use of a broad spectrum of antibodies and elaborate molecular work-up. Further studies for future therapeutic decision making in these newly described round cell sarcomas as well as for...Continue Reading

References

Nov 1, 1994·American Journal of Clinical Pathology·J A LumadueE J Perlman
Nov 24, 1999·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·J P GinsbergF G Barr
Aug 10, 2000·The American Journal of Surgical Pathology·J R GoldblumC Fisher
Feb 15, 2001·The American Journal of Surgical Pathology·P ColliniS Pilotti
Jun 23, 2009·Genome Research·Martin KrzywinskiMarco A Marra
Jul 7, 2009·Nature Cell Biology·Zhipeng FanCun-Yu Wang
Nov 26, 2009·Virchows Archiv : an International Journal of Pathology·Salvatore Romeo, Angelo P Dei Tos
Mar 24, 2010·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Marie-Cecile Le DeleyHeinrich Kovar
Feb 4, 2011·Trends in Molecular Medicine·Silvère M van der MaarelStephen J Tapscott
Aug 6, 2011·Science·Chetan BettegowdaKenneth W Kinzler
Aug 30, 2011·Cancer Genetics·Savita Sankar, Stephen L Lessnick
Mar 6, 2012·Nature Genetics·Gaëlle PierronOlivier Delattre
Jul 7, 2012·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Wei-Lien WangAlexander J Lazar
May 1, 2013·Genes, Chromosomes & Cancer·Ioannis PanagopoulosSverre Heim
Nov 13, 2013·Histopathology·Cristina Antonescu
May 8, 2014·The International Journal of Biochemistry & Cell Biology·Adrián Mariño-Enríquez, Christopher D M Fletcher
May 9, 2014·The American Journal of Surgical Pathology·Florian PulsLars-Gunnar Kindblom
May 27, 2014·Nature Genetics·Xiaoke WangAndré M Oliveira
Jul 10, 2014·The American Journal of Surgical Pathology·Shintaro SugitaTadashi Hasegawa
Jul 18, 2014·Virchows Archiv : an International Journal of Pathology·Ryo ShibuyaMasanori Hisaoka
Oct 17, 2014·The American Journal of Surgical Pathology·David A SolomonMarkku Miettinen
Nov 2, 2014·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Tricia L PetersAngshumoy Roy
Feb 28, 2015·The American Journal of Surgical Pathology·Alberto RighiAngelo Paolo Dei Tos
Jan 12, 2016·The American Journal of Surgical Pathology·Katja SpechtCristina R Antonescu
Feb 6, 2016·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Yin P HungJason L Hornick
Jul 19, 2016·The American Journal of Surgical Pathology·Yu-Chien KaoCristina R Antonescu
Oct 28, 2016·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Yin P HungJason L Hornick
Aug 27, 2016·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Sophie Le GuellecJean-Michel Coindre

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