Document Detail


Impact of EWS-ETS fusion type on disease progression in Ewing's sarcoma/peripheral primitive neuroectodermal tumor: prospective results from the cooperative Euro-E.W.I.N.G. 99 trial.
MedLine Citation:
PMID:  20308673     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
PURPOSE EWS-ETS fusion genes are the driving force in Ewing's sarcoma pathogenesis. Because of the variable breakpoint locations in the involved genes, there is heterogeneity in fusion RNA and protein architecture. Since previous retrospective studies suggested prognostic differences among patients expressing different EWS-FLI1 fusion types, the impact of fusion RNA architecture on disease progression and relapse was studied prospectively within the Euro-E.W.I.N.G. 99 clinical trial. PATIENTS AND METHODS Among 1,957 patients who registered before January 1, 2007, 703 primary tumors were accessible for the molecular biology study. Fusion type was assessed by polymerase chain reaction on frozen (n = 578) or paraffin-embedded materials (n = 125). The primary end point was the time to disease progression or relapse. Results After exclusion of noninformative patients, 565 patients were entered into the prognostic factor analysis comparing type 1 (n = 296), type 2 (n = 133), nontype 1/nontype 2 EWS-FLI1 (n = 91) and EWS-ERG fusions (n = 45). Median follow-up time was 4.5 years. The distribution of sex, age, tumor volume, tumor site, disease extension, or histologic response did not differ between the four fusion type groups. We did not observe any significant prognostic value of the fusion type on the risk of progression or relapse. The only slight difference was that the risk of progression or relapse associated with nontype 1/nontype 2 EWS-FLI1 fusions was 1.38 (95% CI, 0.96 to 2.0) times higher than risk associated with other fusion types, but it was not significant (P = .10). CONCLUSION In contrast to retrospective studies, the prospective evaluation did not confirm a prognostic benefit for type 1 EWS-FLI1 fusions.
Authors:
Marie-Cecile Le Deley; Olivier Delattre; Karl-Ludwig Schaefer; Sue A Burchill; Gabriele Koehler; Pancras C W Hogendoorn; Thomas Lion; Christopher Poremba; Julien Marandet; Stelly Ballet; Gaelle Pierron; Samantha C Brownhill; Michaela Nesslböck; Andreas Ranft; Uta Dirksen; Odile Oberlin; Ian J Lewis; Alan W Craft; Heribert Jürgens; Heinrich Kovar
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Publication Detail:
Type:  Comparative Study; Journal Article; Multicenter Study; Research Support, Non-U.S. Gov't     Date:  2010-03-22
Journal Detail:
Title:  Journal of clinical oncology : official journal of the American Society of Clinical Oncology     Volume:  28     ISSN:  1527-7755     ISO Abbreviation:  J. Clin. Oncol.     Publication Date:  2010 Apr 
Date Detail:
Created Date:  2010-04-19     Completed Date:  2010-04-30     Revised Date:  2014-03-17    
Medline Journal Info:
Nlm Unique ID:  8309333     Medline TA:  J Clin Oncol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1982-8     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Adult
Antineoplastic Combined Chemotherapy Protocols / therapeutic use
Biopsy
Bone Neoplasms / genetics*,  mortality,  pathology,  therapy
Chi-Square Distribution
Disease Progression
Europe
Female
Gene Expression Regulation, Neoplastic*
Genetic Predisposition to Disease
Humans
Kaplan-Meier Estimate
Male
Neoplasm Recurrence, Local
Oncogene Proteins, Fusion / genetics*
Paraffin Embedding
Phenotype
Proportional Hazards Models
Prospective Studies
Proto-Oncogene Protein c-fli-1 / genetics*
RNA-Binding Protein EWS
Radiotherapy, Adjuvant
Reverse Transcriptase Polymerase Chain Reaction
Risk Assessment
Risk Factors
Sarcoma, Ewing / genetics*,  mortality,  secondary,  therapy
Stem Cell Transplantation
Time Factors
Transcription Factors / genetics*
Treatment Outcome
Young Adult
Grant Support
ID/Acronym/Agency:
8176//Cancer Research UK
Chemical
Reg. No./Substance:
0/EWS-ERG fusion protein, human; 0/EWS-FLI fusion protein; 0/Oncogene Proteins, Fusion; 0/Proto-Oncogene Protein c-fli-1; 0/RNA-Binding Protein EWS; 0/Transcription Factors
Comments/Corrections
Comment In:
J Clin Oncol. 2010 Apr 20;28(12):1973-4   [PMID:  20308653 ]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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