UCL Cancer Institute


Published Research

The following academic papers on Ewing sarcoma have been published in peer-reviewed journals. Click on the links to read a summary of each paper.


Therapeutic targeting of the PLK1-PRC1-axis triggers cell death in genomically silent childhood cancer
Jing Li, Shunya Ohmura, Aruna Marchetto, Martin F. Orth, Roland Imle, Marlene Dallmayer, Julian Musa, Maximilian M. L. Knott, Tilman L. B. Hölting, Stefanie Stein, Cornelius M. Funk, Ana Sastre, Javier Alonso, Felix Bestvater, Merve Kasan, Laura Romero-Pérez, Wolfgang Hartmann, Andreas Ranft, Ana Banito, Uta Dirksen, Thomas Kirchner, Florencia Cidre-Aranaz & Thomas G. P. Grünewald

YAP/TAZ inhibition reduces metastatic potential of Ewing sarcoma cells

Lisa Bierbaumer, Anna M. Katschnig, Branka Radic-Sarikas, Maximilian O. Kauer, Jeffrey A. Petro, Sandra Högler, Elisabeth Gurnhofer, Gloria Pedot, Beat W. Schäfer, Raphaela Schwentner, Karin Mühlbacher, Florian Kromp, Dave N. T. Aryee, Lukas Kenner, Aykut Uren, and Heinrich Kovar

Ewing Sarcoma—Diagnosis, Treatment, Clinical Challenges and Future Perspectives

Stefan K. Zöllner, James F. Amatruda, Sebastian Bauer, Stéphane Collaud, Enrique de Álava, Steven G. DuBois, Jendrik Hardes, Wolfgang Hartmann, Heinrich Kovar, Markus Metzler, David S. Shulman, Arne Streitbürger, Beate Timmermann, Jeffrey A. Toretsky, Yasmin Uhlenbruch, Volker Vieth, Thomas G. P. Grünewald, and Uta Dirksen

Multimodal analysis of cell-free DNA whole-genome sequencing for pediatric cancers with low mutational burden

Peter Peneder, Adrian M. Stütz, Didier Surdez, Manuela Krumbholz, Sabine Semper, Mathieu Chicard, Nathan C. Sheffield, Gaelle Pierron, Eve Lapouble, Marcus Tötzl, Bekir Ergüner, Daniele Barreca, André F. Rendeiro, Abbas Agaimy, Heidrun Boztug, Gernot Engstler, Michael Dworzak, Marie Bernkopf, Sabine Taschner-Mandl, Inge M. Ambros, Ola Myklebost, Perrine Marec-Bérard, Susan Ann Burchill, Bernadette Brennan, Sandra J. Strauss, Jeremy Whelan, Gudrun Schleiermacher, Christiane Schaefer, Uta Dirksen, Caroline Hutter, Kjetil Boye, Peter F. Ambros, Olivier Delattre, Markus Metzler, Christoph Bock & Eleni M. Tomazou

"Sequencing of cell-free DNA in the blood of cancer patients (liquid biopsy) provides attractive opportunities for early diagnosis, assessment of treatment response, and minimally invasive disease monitoring." This study included 200 plasma samples from 95 patients with ES, most of whom took part in the EWING2008 trial. 

They showed that "whole-genome sequencing of cfDNA (when combined with suitable computational methods, some of which we developed here) provides a one-size-fits-all assay for liquid biopsy analysis, allowing us to: (i) detect tumor-derived DNA with high sensitivity and without requiring any somatic mutations or CNAs; (ii) distinguish between different cancer types based on their characteristic epigenetic signatures; (iii) monitor CNAs and disease progression over time; (iv) assess treatment-induced toxicity and organ damage based on cfDNA released from dying cells; and (v) estimate survival and relapse probabilities at diagnosis. Importantly, our approach is practically feasible in a clinical setting, requires less than 10 ng of cfDNA, profits from falling sequencing costs, does not require access of primary tumor tissue, and is informative even in the absence of any genetic alterations".


The evolving role of patient advocates in rare cancers: opportunities and challenges
Ornella Gonzato & Alessandro Gronchi


Oncogenic fusion protein EWS-FLI1 is a network hub that regulates alternative splicing
Selvanathan SP, Graham GT, Erkizan HV, Dirksen U, Natarajan TG, Dakic A, Yu S, Liu X, Paulsen MT, Ljungman ME, Wu CH, Lawlor ER, Üren A, Toretsky JA


Blocking the road, stopping the engine or killing the driver? Advances in targeting EWS/FLI-1 fusion in Ewing sarcoma as novel therapy.
Kovar H

Cyclophosphamide Compared With Ifosfamide in Consolidation Treatment of Standard-Risk Ewing Sarcoma: Results of the Randomized Noninferiority Euro-EWING99-R1 Trial
Marie-Cécile Le Deley⇑, Michael Paulussen, Ian Lewis, Bernadette Brennan, Andreas Ranft, Jeremy Whelan, Gwénaël Le Teuff, Jean Michon, Ruth Ladenstein, Perrine Marec-Bérard, Henk van den Berg, Lars Hjorth, Keith Wheatley, Ian Judson, Heribert Juergens, Alan Craft, Odile Oberlin and Uta Dirksen


Interval compressed vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide, etoposide in patients with advanced Ewing’s and other Small Round Cell Sarcomas
Jeremy Whelan, Atia Khan, Anand Sharma, Christian Rothermundt, Palma Dileo, Maria Michelagnoli, Beatrice Seddon, and Sandra Strauss

Randomized controlled trial of interval-compressed chemotherapy for the treatment of localized Ewing sarcoma: a report from the Children's Oncology Group.
Womer RB, West DC, Krailo MD, Dickman PS, Pawel BR, Grier HE, Marcus K, Sailer S, Healey JH, Dormans JP, Weiss AR.


Primary disseminated multifocal Ewing sarcoma: results of the Euro-EWING 99 trial.
Ladenstein R, Pötschger U, Le Deley MC, Whelan J, Paulussen M, Oberlin O, van den Berg H, Dirksen U, Hjorth L, Michon J, Lewis I, Craft A, Jürgens H


Safety assessment of intensive induction with vincristine, ifosfamide, doxorubicin, and etoposide (VIDE) in the treatment of Ewing tumors in the EURO-E.W.I.N.G. 99 clinical trial.
Juergens C, Weston C, Lewis I, Whelan J, Paulussen M, Oberlin O, Michon J, Zoubek A, Juergens H, Craft A


Single Center Experience of a New Intensive Induction Therapy for Ewing’s Family of Tumors: Feasibility, Toxicity, and Stem Cell Mobilization Properties
S.J. Strauss, A. McTiernan, D. Driver, M. Hall-Craggs, A. Sandison, A.M. Cassoni, A. Kilby, M. Michelagnoli, J. Pringle, J. Cobb, T. Briggs, S. Cannon, J. Witt and J.S. Whelan