Desmoplastic small round cell tumors with EWS-WT1 fusion transcript in children and young adults

Background The presence of the EWS‐WT1 gene fusion transcript (GFT) is characteristic of desmoplastic small round cell tumor (DSRCT), a rare and very aggressive disease for which the treatment has not yet been clearly standardized. Methods This was a retrospective national multicenter analysis of yo...

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Veröffentlicht in:	Pediatric blood & cancer 2012-06, Vol.58 (6), p.891-897
Hauptverfasser:	Philippe-Chomette, Pascale, Kabbara, Nabil, Andre, Nicolas, Pierron, Gaelle, Coulomb, Aurore, Laurence, Valérie, Blay, Jean-Yves, Delattre, Olivier, Schleiermacher, Gudrun, Orbach, Daniel
Format:	Artikel
Sprache:	eng
Schlagworte:	Adolescent adolescent-young adult Adult Antineoplastic Combined Chemotherapy Protocols Child Child, Preschool Combined Modality Therapy desmoplastic small round cell tumor Desmoplastic Small Round Cell Tumor - genetics Desmoplastic Small Round Cell Tumor - mortality Desmoplastic Small Round Cell Tumor - surgery Disease-Free Survival EWS-WT1 gene fusion transcript extensive surgery Female high-dose chemotherapy Humans Kaplan-Meier Estimate Male Oncogene Proteins, Fusion - genetics Radiotherapy Real-Time Polymerase Chain Reaction Retrospective Studies Reverse Transcriptase Polymerase Chain Reaction sarcoma Treatment Outcome Young Adult
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Zusammenfassung:	Background The presence of the EWS‐WT1 gene fusion transcript (GFT) is characteristic of desmoplastic small round cell tumor (DSRCT), a rare and very aggressive disease for which the treatment has not yet been clearly standardized. Methods This was a retrospective national multicenter analysis of young patients
ISSN:	1545-5009 1545-5017
DOI:	10.1002/pbc.23403