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...
| Published in: | Pediatric Blood & Cancer Vol. 58; no. 6; pp. 891 - 898 |
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| Main Authors: | , , , , , , , , , |
| Format: | research Journal Article |
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Wiley-Blackwell
Jun2012
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| Online Access: | View this record in EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=104536703&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 104536703 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 15455009 1XL2 jtl: Pediatric Blood & Cancer issn: 15455009 maglogo: Y pubinfo: dt: Jun2012 vid: 58 iid: 6 pid: 480 pub: Wiley-Blackwell place: Malden, Massachusetts artinfo: ui: 104536703 2011493951 10.1002/pbc.23403 NLM22162435 104536703 ppf: 891 ppct: 7 formats: tig: atl: Desmoplastic small round cell tumors with EWS-WT1 fusion transcript in children and young adults. aug: au: Philippe-Chomette P Kabbara N Andre N Pierron G Coulomb A Laurence V Blay JY Delattre O Schleiermacher G Orbach D affil: Department of Pediatric Surgery, Hôpital Robert Debré - Assistance Publique, Paris, France. sug: subj: Sarcoma Sarcoma Mortality Sarcoma Surgery Proteins Adolescence Adult Antineoplastic Agents, Combined Child Child, Preschool Combined Modality Therapy Prognosis Female Human Kaplan-Meier Estimator Male Radiotherapy Polymerase Chain Reaction Retrospective Design Reverse Transcriptase Polymerase Chain Reaction Treatment Outcomes Young Adult Adolescent: 13-18 years Adult: 19-44 years Child: 6-12 years Child, Preschool: 2-5 years Female Male ab: 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 <30 years with tumors expressing the EWS-WT1-GFT, designed to determine whether extensive surgery had an impact on survival. RESULTS: Between 1995 and 2006, a EWS-WT1-GFT was detected in the tumors of 38 patients, 17 (44.7%) of whom had had a different initial pathologic diagnosis prior to molecular testing. Mean age was 13.2 years (range: 4-29.7 years). Only 9 patients (24%) had localized disease. Treatment was heterogeneous. Nine patients had 'limited' surgical resections and 22 underwent 'extensive' surgery. Two-year event-free survival and overall survival were 14.4% and 50%, respectively. Among the five patients who were alive in complete remission, four had undergone extensive and complete surgery. CONCLUSIONS: Detection of the EWS-WT1-GFT plays a major role in the diagnosis of DSRCT. No survival difference was observed according to extent of surgery, but complete surgery seemed to offer the best chance of long-term survival. High-dose chemotherapy or local radiotherapy did not appear to improve survival in this retrospective analysis, but larger prospective studies are needed to provide definitive conclusions on the role of these treatments. Pediatr Blood Cancer 2012; 58: 891-897. © 2011 Wiley Periodicals, Inc. pubtype: Academic Journal doctype: research Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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