Prognostic value of PAX–FKHR fusion status in alveolar rhabdomyosarcoma: A report from the cooperative soft tissue sarcoma study group (CWS)
Identifieur interne : 000D80 ( Istex/Corpus ); précédent : 000D79; suivant : 000D81Prognostic value of PAX–FKHR fusion status in alveolar rhabdomyosarcoma: A report from the cooperative soft tissue sarcoma study group (CWS)
Auteurs : Sabine Stegmaier ; Christopher Poremba ; Karl-Ludwig Schaefer ; Ivo Leuschner ; Bernarda Kazanowska ; Albert N. Békássy ; Stefan S. Bielack ; Thomas Klingebiel ; Ewa KoscielniakSource :
- Pediatric Blood & Cancer [ 1545-5009 ] ; 2011-09.
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Abstract
Alveolar Rhabdomyosarcomas (RMA) are characterized by chromosomal translocations, fusing the PAX3 or PAX7 gene with FKHR in about 85%. Previous studies have suggested that the fusion type is associated with prognosis. In order to investigate the predictive value of the PAX–FKHR fusion status on disease outcome of patients with RMA treated in the CWS trials we performed a retrospective analysis.
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DOI: 10.1002/pbc.22958
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<record><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Prognostic value of PAX–FKHR fusion status in alveolar rhabdomyosarcoma: A report from the cooperative soft tissue sarcoma study group (CWS)</title><author><name sortKey="Stegmaier, Sabine" sort="Stegmaier, Sabine" uniqKey="Stegmaier S" first="Sabine" last="Stegmaier">Sabine Stegmaier</name><affiliation><mods:affiliation>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Stuttgart, Germany</mods:affiliation></affiliation><affiliation><mods:affiliation>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Bismarckstr. 8, 70176 Stuttgart, Germany.===</mods:affiliation></affiliation></author><author><name sortKey="Poremba, Christopher" sort="Poremba, Christopher" uniqKey="Poremba C" first="Christopher" last="Poremba">Christopher Poremba</name><affiliation><mods:affiliation>Institute of Pathology, Heinrich‐Heine‐University, Duesseldorf, Germany</mods:affiliation></affiliation><affiliation><mods:affiliation>Center of Histopathology, Cytology & Molecular Diagnostics (CHCMD), Research Park Trier, Trier, Germany</mods:affiliation></affiliation></author><author><name sortKey="Schaefer, Karl Udwig" sort="Schaefer, Karl Udwig" uniqKey="Schaefer K" first="Karl-Ludwig" last="Schaefer">Karl-Ludwig Schaefer</name><affiliation><mods:affiliation>Institute of Pathology, Heinrich‐Heine‐University, Duesseldorf, Germany</mods:affiliation></affiliation></author><author><name sortKey="Leuschner, Ivo" sort="Leuschner, Ivo" uniqKey="Leuschner I" first="Ivo" last="Leuschner">Ivo Leuschner</name><affiliation><mods:affiliation>Department of Pathology, Institute for Pediatric Pathology, Kiel Pediatric Tumor Registry, University of Kiel, Kiel, Germany</mods:affiliation></affiliation></author><author><name sortKey="Kazanowska, Bernarda" sort="Kazanowska, Bernarda" uniqKey="Kazanowska B" first="Bernarda" last="Kazanowska">Bernarda Kazanowska</name><affiliation><mods:affiliation>Department of Bone Marrow Transplantation, Pediatric Oncology and Hematology, University of Medicine, Wroclaw, Poland</mods:affiliation></affiliation></author><author><name sortKey="Bekassy, Albert N" sort="Bekassy, Albert N" uniqKey="Bekassy A" first="Albert N." last="Békássy">Albert N. 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Békássy</name><affiliation><mods:affiliation>Department of Pediatric Oncology‐Hematology, University Children's Hospital, Lund, Sweden</mods:affiliation></affiliation></author><author><name sortKey="Bielack, Stefan S" sort="Bielack, Stefan S" uniqKey="Bielack S" first="Stefan S." last="Bielack">Stefan S. Bielack</name><affiliation><mods:affiliation>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Stuttgart, Germany</mods:affiliation></affiliation><affiliation><mods:affiliation>Department of Pediatric Hematology and Oncology, University Childrens' Hospital Muenster, Muenster, Germany</mods:affiliation></affiliation></author><author><name sortKey="Klingebiel, Thomas" sort="Klingebiel, Thomas" uniqKey="Klingebiel T" first="Thomas" last="Klingebiel">Thomas Klingebiel</name><affiliation><mods:affiliation>Department of Pediatric Oncology, University of Frankfurt (Main), Frankfurt, Germany</mods:affiliation></affiliation></author><author><name sortKey="Koscielniak, Ewa" sort="Koscielniak, Ewa" uniqKey="Koscielniak E" first="Ewa" last="Koscielniak">Ewa Koscielniak</name><affiliation><mods:affiliation>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Stuttgart, Germany</mods:affiliation></affiliation></author></analytic><monogr></monogr><series><title level="j">Pediatric Blood & Cancer</title><title level="j" type="abbrev">Pediatr. Blood Cancer</title><idno type="ISSN">1545-5009</idno><idno type="eISSN">1545-5017</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2011-09">2011-09</date><biblScope unit="volume">57</biblScope><biblScope unit="issue">3</biblScope><biblScope unit="page" from="406">406</biblScope><biblScope unit="page" to="414">414</biblScope></imprint><idno type="ISSN">1545-5009</idno></series><idno type="istex">3F2A4A97541A975486E92F1C020E9BEE07E6F707</idno><idno type="DOI">10.1002/pbc.22958</idno><idno type="ArticleID">PBC22958</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">1545-5009</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>PAX–FKHR</term><term>RT‐PCR</term><term>prognosis</term><term>rhabdomyosarcoma</term><term>translocation</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract">Alveolar Rhabdomyosarcomas (RMA) are characterized by chromosomal translocations, fusing the PAX3 or PAX7 gene with FKHR in about 85%. Previous studies have suggested that the fusion type is associated with prognosis. In order to investigate the predictive value of the PAX–FKHR fusion status on disease outcome of patients with RMA treated in the CWS trials we performed a retrospective analysis.</div></front></TEI><istex><corpusName>wiley</corpusName><author><json:item><name>Sabine Stegmaier ScD</name><affiliations><json:string>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Stuttgart, Germany</json:string><json:string>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Bismarckstr. 8, 70176 Stuttgart, Germany.===</json:string></affiliations></json:item><json:item><name>Christopher Poremba MD</name><affiliations><json:string>Institute of Pathology, Heinrich‐Heine‐University, Duesseldorf, Germany</json:string><json:string>Center of Histopathology, Cytology & Molecular Diagnostics (CHCMD), Research Park Trier, Trier, Germany</json:string></affiliations></json:item><json:item><name>Karl‐Ludwig Schaefer ScD</name><affiliations><json:string>Institute of Pathology, Heinrich‐Heine‐University, Duesseldorf, Germany</json:string></affiliations></json:item><json:item><name>Ivo Leuschner MD</name><affiliations><json:string>Department of Pathology, Institute for Pediatric Pathology, Kiel Pediatric Tumor Registry, University of Kiel, Kiel, Germany</json:string></affiliations></json:item><json:item><name>Bernarda Kazanowska MD</name><affiliations><json:string>Department of Bone Marrow Transplantation, Pediatric Oncology and Hematology, University of Medicine, Wroclaw, Poland</json:string></affiliations></json:item><json:item><name>Albert N. Békássy MD</name><affiliations><json:string>Department of Pediatric Oncology‐Hematology, University Children's Hospital, Lund, Sweden</json:string></affiliations></json:item><json:item><name>Stefan S. Bielack MD</name><affiliations><json:string>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Stuttgart, Germany</json:string><json:string>Department of Pediatric Hematology and Oncology, University Childrens' Hospital Muenster, Muenster, Germany</json:string></affiliations></json:item><json:item><name>Thomas Klingebiel MD</name><affiliations><json:string>Department of Pediatric Oncology, University of Frankfurt (Main), Frankfurt, Germany</json:string></affiliations></json:item><json:item><name>Ewa Koscielniak MD</name><affiliations><json:string>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Stuttgart, Germany</json:string></affiliations></json:item></author><subject><json:item><lang><json:string>eng</json:string></lang><value>PAX–FKHR</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>prognosis</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>rhabdomyosarcoma</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>RT‐PCR</value></json:item><json:item><lang><json:string>eng</json:string></lang><value>translocation</value></json:item></subject><articleId><json:string>PBC22958</json:string></articleId><language><json:string>eng</json:string></language><originalGenre><json:string>article</json:string></originalGenre><qualityIndicators><score>5.054</score><pdfVersion>1.3</pdfVersion><pdfPageSize>594 x 792 pts</pdfPageSize><refBibsNative>true</refBibsNative><abstractCharCount>397</abstractCharCount><pdfWordCount>4334</pdfWordCount><pdfCharCount>29971</pdfCharCount><pdfPageCount>9</pdfPageCount><abstractWordCount>60</abstractWordCount></qualityIndicators><title>Prognostic value of PAX–FKHR fusion status in alveolar rhabdomyosarcoma: A report from the cooperative soft tissue sarcoma study group (CWS)</title><refBibs><json:item><host><volume>41</volume><issue>6</issue><author></author><title>German Childhood Cancer Registry—Annual Report 2005 (1980–2004). Mainz, Germany, Deutsches Kinderkrebsregister, 2005</title></host></json:item><json:item><author><json:item><name>T Weihkopf</name></json:item><json:item><name>M Blettner</name></json:item><json:item><name>T Dantonello</name></json:item></author><host><volume>44</volume><pages><last>440</last><first>432</first></pages><author></author><title>Eur J Cancer</title></host><title>Incidence and time trends of soft tissue sarcomas in German children 1985–2004—A report from the population‐based German Childhood Cancer Registry</title></json:item><json:item><author><json:item><name>E Koscielniak</name></json:item><json:item><name>D Harms</name></json:item><json:item><name>G Henze</name></json:item></author><host><volume>17</volume><pages><last>3719</last><first>3706</first></pages><author></author><title>J Clin Oncol</title></host><title>Results of treatment for soft tissue sarcoma in childhood and adolescence: A final report of the German Cooperative Soft Tissue Sarcoma Study CWS‐86</title></json:item><json:item><author><json:item><name>N Galili</name></json:item><json:item><name>RJ Davis</name></json:item><json:item><name>WJ Fredericks</name></json:item></author><host><volume>5</volume><pages><last>235</last><first>230</first></pages><author></author><title>Nat Genet</title></host><title>Fusion of a fork head domain gene to PAX3 in the solid tumour alveolar rhabdomyosarcoma</title></json:item><json:item><author><json:item><name>RJ Davis</name></json:item><json:item><name>CM D'Cruz</name></json:item><json:item><name>MA Lovell</name></json:item></author><host><volume>54</volume><pages><last>2872</last><first>2869</first></pages><author></author><title>Cancer Res</title></host><title>Fusion of PAX7 to FKHR by the variant t(1;13)(p36;q14) translocation in alveolar rhabdomyosarcoma</title></json:item><json:item><author><json:item><name>WJ Fredericks</name></json:item><json:item><name>N Galili</name></json:item><json:item><name>S Mukhopadhyay</name></json:item></author><host><volume>15</volume><pages><last>1535</last><first>1522</first></pages><author></author><title>Mol Cell Biol</title></host><title>The PAX3–FKHR fusion protein created by the t(2;13) translocation in alveolar rhabdomyosarcomas is a more potent transcriptional activator than PAX3</title></json:item><json:item><author><json:item><name>J Anderson</name></json:item><json:item><name>T Gordon</name></json:item><json:item><name>A McManus</name></json:item></author><host><volume>85</volume><pages><last>835</last><first>831</first></pages><author></author><title>Br J Cancer</title></host><title>Detection of the PAX3–FKHR fusion gene in paediatric rhabdomyosarcoma: A reproducible predictor of outcome</title></json:item><json:item><author><json:item><name>KM Kelly</name></json:item><json:item><name>RB Wormer</name></json:item><json:item><name>PH Sorensen</name></json:item></author><host><volume>15</volume><pages><last>1836</last><first>1831</first></pages><author></author><title>J Clin Oncol</title></host><title>Common and variant gene fusions predict distinct clinical phenotypes in rhabdomyosarcoma</title></json:item><json:item><author><json:item><name>PH Sorensen</name></json:item><json:item><name>JC Lynch</name></json:item><json:item><name>SJ Qualman</name></json:item></author><host><volume>20</volume><pages><last>2679</last><first>2672</first></pages><author></author><title>J Clin Oncol</title></host><title>PAX3–FKHR and PAX7–FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: A report from the children's oncology group</title></json:item><json:item><author><json:item><name>B Kazanowska</name></json:item><json:item><name>A Reich</name></json:item><json:item><name>S Stegmaier</name></json:item></author><host><volume>26</volume><pages><last>31</last><first>17</first></pages><author></author><title>Fetal Pediatr Pathol</title></host><title>PAX3–FKHR and PAX7–FKHR fusion genes impact outcome of alveolar rhabdomyosarcoma in children</title></json:item><json:item><author><json:item><name>E Davicioni</name></json:item><json:item><name>FG Finckenstein</name></json:item><json:item><name>V Shahbazian</name></json:item></author><host><volume>66</volume><pages><last>6946</last><first>6936</first></pages><author></author><title>Cancer Res</title></host><title>Identification of a PAX–FKHR gene expression signature that defines molecular classes and determines the prognosis of alveolar rhabdomyosarcomas</title></json:item><json:item><author><json:item><name>E Davicioni</name></json:item><json:item><name>JR Anderson</name></json:item><json:item><name>JD Buckley</name></json:item></author><host><volume>28</volume><pages><last>1246</last><first>1240</first></pages><author></author><title>J Clin Oncol</title></host><title>Gene expression profiling for survival prediction in pediatric rhabdomyosarcomas: A report from the Children's Oncology Group</title></json:item><json:item><author><json:item><name>D Schmidt</name></json:item><json:item><name>D Harms</name></json:item><json:item><name>O Reimann</name></json:item></author><host><volume>198</volume><pages><last>207</last><first>202</first></pages><author></author><title>Klein Padiatr</title></host><title>Rhabdomyosarkom: Morphologie und zelluläre Differenzierung</title></json:item><json:item><author><json:item><name>TM Dantonello</name></json:item><json:item><name>C Int‐Veen</name></json:item><json:item><name>D Harms</name></json:item></author><host><volume>27</volume><pages><last>1455</last><first>1446</first></pages><author></author><title>J Clin Oncol</title></host><title>Cooperative trial CWS‐91 for localized soft tissue sarcoma in children, adolescents, and young adults</title></json:item><json:item><author><json:item><name>D Modritz</name></json:item><json:item><name>R Ladenstein</name></json:item><json:item><name>U Potschger</name></json:item></author><host><volume>117</volume><pages><last>209</last><first>196</first></pages><author></author><title>Wien Klin Wochenschr</title></host><title>Treatment for soft tissue sarcoma in childhood and adolescence. Austrian results within the CWS 96 study</title></json:item><json:item><author><json:item><name>M Carli</name></json:item><json:item><name>E Koscielniak</name></json:item><json:item><name>G Bisogno</name></json:item></author><host><volume>9</volume><pages><first> 053</first></pages><author></author><title>Sarcoma</title></host><title>ICG‐CWS RMS96: Early results of the Italian an German Rhabdomyosarcoma (RMS) Cooperative Study</title></json:item><json:item><author><json:item><name>W Lawrence Jr.</name></json:item><json:item><name>EA Gehan</name></json:item><json:item><name>DM Hays</name></json:item></author><host><volume>5</volume><pages><last>54</last><first>46</first></pages><author></author><title>J Clin Oncol</title></host><title>Prognostic significance of staging factors of the UICC staging system in childhood rhabdomyosarcoma: A report from the Intergroup Rhabdomyosarcoma Study (IRS‐II)</title></json:item><json:item><host><volume>41</volume><pages><last>28</last><first>23</first></pages><issue>6</issue><author></author><title>Harmer MH. TNM classification of pediatric tumors. Geneva: Union International contre le Cancer; 1982. pp. 23–28.</title></host></json:item><json:item><author><json:item><name>C Rodary</name></json:item><json:item><name>F Flamant</name></json:item><json:item><name>SS Donaldson</name></json:item></author><host><volume>17</volume><pages><last>215</last><first>210</first></pages><author></author><title>Med Pediatr Oncol</title></host><title>An attempt to use a common staging system in rhabdomyosarcoma: A report of an international workshop initiated by the International Society of Pediatric Oncology (SIOP)</title></json:item><json:item><author><json:item><name>C Poremba</name></json:item><json:item><name>C Scheel</name></json:item><json:item><name>B Hero</name></json:item></author><host><volume>18</volume><pages><last>2592</last><first>2582</first></pages><author></author><title>J Clin Oncol</title></host><title>Telomerase activity and telomerase subunits gene expression patterns in neuroblastoma: A molecular and immunohistochemical study establishing prognostic tools for fresh‐frozen and paraffin‐embedded tissues</title></json:item><json:item><author><json:item><name>S Stegmaier</name></json:item><json:item><name>I Leuschner</name></json:item><json:item><name>E Aakcha‐Rudel</name></json:item></author><host><volume>216</volume><pages><last>322</last><first>315</first></pages><author></author><title>Klin Padiatr</title></host><title>Identification of various exon combinations of the ews/fli1 translocation: An optimized RT‐PCR method for paraffin embedded tissue—A report by the CWS‐study group</title></json:item><json:item><author><json:item><name>B Dockhorn‐Dworniczak</name></json:item><json:item><name>KL Schafer</name></json:item><json:item><name>S Blasius</name></json:item></author><host><volume>209</volume><pages><last>164</last><first>156</first></pages><author></author><title>Klin Padiatr</title></host><title>Assessment of molecular genetic detection of chromosome translocations in the differential diagnosis of pediatric sarcomas</title></json:item><json:item><author><json:item><name>El Kaplan</name></json:item><json:item><name>P Meier</name></json:item></author><host><volume>53</volume><pages><last>481</last><first>457</first></pages><author></author><title>J Am Stat Assoc</title></host><title>Nonparametric estimation from incomplete observations</title></json:item><json:item><host><volume>41</volume><pages><last>26</last><first>1</first></pages><issue>6</issue><author></author><title>Greenwood M. The natural duration of cancer. Reports on public health and medical subjects. Vol. 33. London: Her Majesty's Stationery Office; 1926. pp. 1–26.</title></host></json:item><json:item><author><json:item><name>R Peto</name></json:item><json:item><name>MC Pike</name></json:item><json:item><name>P Armitage</name></json:item></author><host><volume>35</volume><pages><last>39</last><first>1</first></pages><author></author><title>Br J Cancer</title></host><title>Design and analysis of randomized clinical trials requiring prolonged observation of each patient: II. Analysis and examples</title></json:item><json:item><author><json:item><name>DR Cox</name></json:item></author><host><volume>34</volume><pages><last>220</last><first>187</first></pages><author></author><title>J R Stat Soc B</title></host><title>Regression models and life‐tables</title></json:item><json:item><author><json:item><name>M Lae</name></json:item><json:item><name>EH Ahn</name></json:item><json:item><name>GE Mercado</name></json:item></author><host><volume>212</volume><pages><last>151</last><first>143</first></pages><author></author><title>J Pathol</title></host><title>Global gene expression profiling of PAX–FKHR fusion positive alveolar and PAX–FKHR fusion‐negative embryonal rhabdomyosarcomas</title></json:item><json:item><author><json:item><name>M Wachtel</name></json:item><json:item><name>M Dettling</name></json:item><json:item><name>E Koscielniak</name></json:item></author><host><volume>64</volume><pages><last>5545</last><first>5539</first></pages><author></author><title>Cancer Res</title></host><title>Gene expression signatures identify rhabdomyosarcoma subtypes and detect a novel t(2;2)(q35;p23) translocation fusing PAX3 to NCOA1</title></json:item><json:item><author><json:item><name>D Williamson</name></json:item><json:item><name>E Missiaglia</name></json:item><json:item><name>A de Reyniès</name></json:item></author><host><volume>28</volume><pages><last>2158</last><first>2151</first></pages><author></author><title>J Clin Oncol</title></host><title>Fusion gene‐negative alveolar rhabdomyosarcoma is clinically and molecularly indistinguishable from embryonal rhabdomyosarcoma</title></json:item><json:item><author><json:item><name>FG Barr</name></json:item></author><host><volume>20</volume><pages><last>5746</last><first>5736</first></pages><author></author><title>Oncogene</title></host><title>Gene fusions involving PAX and FOX family members in alveolar rhabdomyosarcoma</title></json:item><json:item><author><json:item><name>FG Barr</name></json:item><json:item><name>LM Smith</name></json:item><json:item><name>JC Lynch</name></json:item></author><host><volume>8</volume><pages><last>208</last><first>202</first></pages><author></author><title>J Mol Diagn</title></host><title>Examination of gene fusions in archival samples of alveolar rhabdomyosarcoma entered on the intergroup rhabdomyosarcoma study‐III trial</title></json:item><json:item><author><json:item><name>W Crist</name></json:item><json:item><name>EA Gehan</name></json:item><json:item><name>AH Ragab</name></json:item></author><host><volume>13</volume><pages><last>630</last><first>610</first></pages><author></author><title>J Clin Oncol</title></host><title>The Third Intergroup Rhabdomyosarcoma Study</title></json:item><json:item><author><json:item><name>WM Crist</name></json:item><json:item><name>JR Anderson</name></json:item><json:item><name>JL Meza</name></json:item></author><host><volume>19</volume><pages><last>3102</last><first>3091</first></pages><author></author><title>J Clin Oncol</title></host><title>Intergroup rhabdomyosarcoma study‐IV: Results for patients with nonmetastatic disease</title></json:item><json:item><author><json:item><name>TM Dantonello</name></json:item><json:item><name>C Int‐Veen</name></json:item><json:item><name>P Winkler</name></json:item></author><host><volume>26</volume><pages><last>413</last><first>406</first></pages><author></author><title>J Clin Oncol</title></host><title>Initial patient characteristics can predict pattern and risk of relapse in localized Rhabdomyosarcoma</title></json:item><json:item><author><json:item><name>M Wachtel</name></json:item><json:item><name>T Runge</name></json:item><json:item><name>I Leuschner</name></json:item></author><host><volume>24</volume><pages><last>822</last><first>816</first></pages><author></author><title>J Clin Oncol</title></host><title>Subtype and prognostic classification of rhabdomyosarcoma by immunohistochemistry</title></json:item><json:item><author><json:item><name>FG Barr</name></json:item><json:item><name>SJ Qualman</name></json:item><json:item><name>MH Macris</name></json:item></author><host><volume>62</volume><pages><last>4710</last><first>4704</first></pages><author></author><title>Cancer Res</title></host><title>Genetic heterogeneity in the alveolar rhabdomyosarcoma subset without typical gene fusions</title></json:item></refBibs><genre><json:string>article</json:string></genre><host><volume>57</volume><publisherId><json:string>PBC</json:string></publisherId><pages><total>9</total><last>414</last><first>406</first></pages><issn><json:string>1545-5009</json:string></issn><issue>3</issue><subject><json:item><value>Research Article</value></json:item></subject><genre><json:string>journal</json:string></genre><language><json:string>unknown</json:string></language><eissn><json:string>1545-5017</json:string></eissn><title>Pediatric Blood & Cancer</title><doi><json:string>10.1002/(ISSN)1545-5017</json:string></doi></host><publicationDate>2011</publicationDate><copyrightDate>2011</copyrightDate><doi><json:string>10.1002/pbc.22958</json:string></doi><id>3F2A4A97541A975486E92F1C020E9BEE07E6F707</id><score>0.22631137</score><fulltext><json:item><extension>pdf</extension><original>true</original><mimetype>application/pdf</mimetype><uri>https://api.istex.fr/document/3F2A4A97541A975486E92F1C020E9BEE07E6F707/fulltext/pdf</uri></json:item><json:item><extension>zip</extension><original>false</original><mimetype>application/zip</mimetype><uri>https://api.istex.fr/document/3F2A4A97541A975486E92F1C020E9BEE07E6F707/fulltext/zip</uri></json:item><istex:fulltextTEI uri="https://api.istex.fr/document/3F2A4A97541A975486E92F1C020E9BEE07E6F707/fulltext/tei"><teiHeader><fileDesc><titleStmt><title level="a" type="main" xml:lang="en">Prognostic value of PAX–FKHR fusion status in alveolar rhabdomyosarcoma: A report from the cooperative soft tissue sarcoma study group (CWS)</title></titleStmt><publicationStmt><authority>ISTEX</authority><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><availability><p>Copyright © 2011 Wiley‐Liss, Inc.</p></availability><date>2011</date></publicationStmt><notesStmt><note type="content">*Conflict of Interest: Nothing to report.</note><note>Foerderkreis Krebskranke Kinder e.V., Stuttgart, Germany</note><note>Olgaele Stiftung für das kranke Kind e.V., Stuttgart, Germany</note><note>Deutsche Krebshilfe e.V., Bonn, Germany - No. 50‐2721;</note></notesStmt><sourceDesc><biblStruct type="inbook"><analytic><title level="a" type="main" xml:lang="en">Prognostic value of PAX–FKHR fusion status in alveolar rhabdomyosarcoma: A report from the cooperative soft tissue sarcoma study group (CWS)</title><author xml:id="author-1"><persName><forename type="first">Sabine</forename><surname>Stegmaier</surname></persName><roleName type="degree">ScD</roleName><affiliation>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Stuttgart, Germany</affiliation><affiliation>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Bismarckstr. 8, 70176 Stuttgart, Germany.===</affiliation></author><author xml:id="author-2"><persName><forename type="first">Christopher</forename><surname>Poremba</surname></persName><roleName type="degree">MD</roleName><affiliation>Institute of Pathology, Heinrich‐Heine‐University, Duesseldorf, Germany</affiliation><affiliation>Center of Histopathology, Cytology & Molecular Diagnostics (CHCMD), Research Park Trier, Trier, Germany</affiliation></author><author xml:id="author-3"><persName><forename type="first">Karl‐Ludwig</forename><surname>Schaefer</surname></persName><roleName type="degree">ScD</roleName><affiliation>Institute of Pathology, Heinrich‐Heine‐University, Duesseldorf, Germany</affiliation></author><author xml:id="author-4"><persName><forename type="first">Ivo</forename><surname>Leuschner</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Pathology, Institute for Pediatric Pathology, Kiel Pediatric Tumor Registry, University of Kiel, Kiel, Germany</affiliation></author><author xml:id="author-5"><persName><forename type="first">Bernarda</forename><surname>Kazanowska</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Bone Marrow Transplantation, Pediatric Oncology and Hematology, University of Medicine, Wroclaw, Poland</affiliation></author><author xml:id="author-6"><persName><forename type="first">Albert N.</forename><surname>Békássy</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Pediatric Oncology‐Hematology, University Children's Hospital, Lund, Sweden</affiliation></author><author xml:id="author-7"><persName><forename type="first">Stefan S.</forename><surname>Bielack</surname></persName><roleName type="degree">MD</roleName><affiliation>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Stuttgart, Germany</affiliation><affiliation>Department of Pediatric Hematology and Oncology, University Childrens' Hospital Muenster, Muenster, Germany</affiliation></author><author xml:id="author-8"><persName><forename type="first">Thomas</forename><surname>Klingebiel</surname></persName><roleName type="degree">MD</roleName><affiliation>Department of Pediatric Oncology, University of Frankfurt (Main), Frankfurt, Germany</affiliation></author><author xml:id="author-9"><persName><forename type="first">Ewa</forename><surname>Koscielniak</surname></persName><roleName type="degree">MD</roleName><affiliation>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Stuttgart, Germany</affiliation></author></analytic><monogr><title level="j">Pediatric Blood & Cancer</title><title level="j" type="abbrev">Pediatr. Blood Cancer</title><idno type="pISSN">1545-5009</idno><idno type="eISSN">1545-5017</idno><idno type="DOI">10.1002/(ISSN)1545-5017</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2011-09"></date><biblScope unit="volume">57</biblScope><biblScope unit="issue">3</biblScope><biblScope unit="page" from="406">406</biblScope><biblScope unit="page" to="414">414</biblScope></imprint></monogr><idno type="istex">3F2A4A97541A975486E92F1C020E9BEE07E6F707</idno><idno type="DOI">10.1002/pbc.22958</idno><idno type="ArticleID">PBC22958</idno></biblStruct></sourceDesc></fileDesc><profileDesc><creation><date>2011</date></creation><langUsage><language ident="en">en</language></langUsage><abstract><p>Alveolar Rhabdomyosarcomas (RMA) are characterized by chromosomal translocations, fusing the PAX3 or PAX7 gene with FKHR in about 85%. Previous studies have suggested that the fusion type is associated with prognosis. In order to investigate the predictive value of the PAX–FKHR fusion status on disease outcome of patients with RMA treated in the CWS trials we performed a retrospective analysis.</p></abstract><abstract><p>Between 1986 and 2004, out of 446 patients with RMA treated in four consecutive CWS trials, tumor samples from 126 patients were available for RT‐PCR analysis. Survival depending on fusion status in context with known clinical risk‐factors was analyzed.</p></abstract><abstract><p>Out of 126 samples, 121 had adequate quality for PAX–FKHR fusion status analysis. PAX–FKHR fusions were detected in 101 samples: 60% PAX3–FKHR and 24% PAX7–FKHR fusions, 17% were fusion‐negative. There was no significant difference in survival between patients with PAX3–FKHR versus PAX7–FKHR positive tumors. The fusion transcript negative cohort showed a more favorable outcome than the fusion transcript positive cohort among patients with metastatic disease. From the established clinical risk‐factors none was associated with a significantly higher risk of failure or death in a multivariate analysis.</p></abstract><abstract><p>PAX–FKHR fusion type was not a significant predictor for survival in our analysis. More extensive molecular analyses are needed to identify features with prognostic relevance and useful therapeutic impact. Pediatr Blood Cancer 2011; 57: 406–414. © 2011 Wiley‐Liss, Inc.</p></abstract><textClass xml:lang="en"><keywords scheme="keyword"><list><head>keywords</head><item><term>PAX–FKHR</term></item><item><term>prognosis</term></item><item><term>rhabdomyosarcoma</term></item><item><term>RT‐PCR</term></item><item><term>translocation</term></item></list></keywords></textClass><textClass><keywords scheme="Journal Subject"><list><head>article-category</head><item><term>Research Article</term></item></list></keywords></textClass></profileDesc><revisionDesc><change when="2009-12-15">Received</change><change when="2010-11-10">Registration</change><change when="2011-09">Published</change></revisionDesc></teiHeader></istex:fulltextTEI><json:item><extension>txt</extension><original>false</original><mimetype>text/plain</mimetype><uri>https://api.istex.fr/document/3F2A4A97541A975486E92F1C020E9BEE07E6F707/fulltext/txt</uri></json:item></fulltext><metadata><istex:metadataXml wicri:clean="Wiley, elements deleted: body"><istex:xmlDeclaration>version="1.0" encoding="UTF-8" standalone="yes"</istex:xmlDeclaration><istex:document><component version="2.0" type="serialArticle" xml:lang="en"><header><publicationMeta level="product"><publisherInfo><publisherName>Wiley Subscription Services, Inc., A Wiley Company</publisherName><publisherLoc>Hoboken</publisherLoc></publisherInfo><doi registered="yes">10.1002/(ISSN)1545-5017</doi><issn type="print">1545-5009</issn><issn type="electronic">1545-5017</issn><idGroup><id type="product" value="PBC"></id></idGroup><titleGroup><title type="main" xml:lang="en" sort="PEDIATRIC BLOOD AND CANCER">Pediatric Blood & Cancer</title><title type="short">Pediatr. Blood Cancer</title></titleGroup><selfCitationGroup><citation type="ancestor" xml:id="cit1"><journalTitle>Medical and Pediatric Oncology</journalTitle><accessionId ref="info:x-wiley/issn/00960802">0096-0802</accessionId><accessionId ref="info:x-wiley/issn/1096911X">1096-911X</accessionId><pubYear year="2003">2003</pubYear><vol>41</vol><issue>6</issue></citation></selfCitationGroup></publicationMeta><publicationMeta level="part" position="30"><doi origin="wiley" registered="yes">10.1002/pbc.v57.3</doi><numberingGroup><numbering type="journalVolume" number="57">57</numbering><numbering type="journalIssue">3</numbering></numberingGroup><coverDate startDate="2011-09">September 2011</coverDate></publicationMeta><publicationMeta level="unit" type="article" position="100" status="forIssue"><doi origin="wiley" registered="yes">10.1002/pbc.22958</doi><idGroup><id type="unit" value="PBC22958"></id></idGroup><countGroup><count type="pageTotal" number="9"></count></countGroup><titleGroup><title type="articleCategory">Research Article</title><title type="tocHeading1">Research Articles</title></titleGroup><copyright ownership="publisher">Copyright © 2011 Wiley‐Liss, Inc.</copyright><eventGroup><event type="manuscriptReceived" date="2009-12-15"></event><event type="manuscriptAccepted" date="2010-11-10"></event><event type="xmlConverted" agent="Converter:JWSART34_TO_WML3G version:2.5.2 mode:FullText" date="2011-07-08"></event><event type="publishedOnlineEarlyUnpaginated" date="2011-01-19"></event><event type="firstOnline" date="2011-01-19"></event><event type="publishedOnlineFinalForm" date="2011-07-08"></event><event type="xmlConverted" agent="Converter:WILEY_ML3G_TO_WILEY_ML3GV2 version:4.0.1" date="2014-03-20"></event><event type="xmlConverted" agent="Converter:WML3G_To_WML3G version:4.1.7 mode:FullText,remove_FC" date="2014-11-03"></event></eventGroup><numberingGroup><numbering type="pageFirst">406</numbering><numbering type="pageLast">414</numbering></numberingGroup><correspondenceTo>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Bismarckstr. 8, 70176 Stuttgart, Germany.===</correspondenceTo><objectNameGroup><objectName elementName="appendix">APPENDIX</objectName></objectNameGroup><linkGroup><link type="toTypesetVersion" href="file:PBC.PBC22958.pdf"></link></linkGroup></publicationMeta><contentMeta><countGroup><count type="figureTotal" number="2"></count><count type="tableTotal" number="3"></count><count type="referenceTotal" number="36"></count><count type="wordTotal" number="6401"></count></countGroup><titleGroup><title type="main" xml:lang="en">Prognostic value of PAX–FKHR fusion status in alveolar rhabdomyosarcoma: A report from the cooperative soft tissue sarcoma study group (CWS)<link href="#fn1"></link></title><title type="short" xml:lang="en">PAX–FKHR and Prognosis of RMA</title></titleGroup><creators><creator xml:id="au1" creatorRole="author" affiliationRef="#af1" corresponding="yes"><personName><givenNames>Sabine</givenNames><familyName>Stegmaier</familyName><degrees>ScD</degrees></personName><contactDetails><email normalForm="s.stegmaier@klinikum-stuttgart.de">s.stegmaier@klinikum‐stuttgart.de</email></contactDetails></creator><creator xml:id="au2" creatorRole="author" affiliationRef="#af2 #af3"><personName><givenNames>Christopher</givenNames><familyName>Poremba</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au3" creatorRole="author" affiliationRef="#af2"><personName><givenNames>Karl‐Ludwig</givenNames><familyName>Schaefer</familyName><degrees>ScD</degrees></personName></creator><creator xml:id="au4" creatorRole="author" affiliationRef="#af4"><personName><givenNames>Ivo</givenNames><familyName>Leuschner</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au5" creatorRole="author" affiliationRef="#af5"><personName><givenNames>Bernarda</givenNames><familyName>Kazanowska</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au6" creatorRole="author" affiliationRef="#af6"><personName><givenNames>Albert N.</givenNames><familyName>Békássy</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au7" creatorRole="author" affiliationRef="#af1 #af7"><personName><givenNames>Stefan S.</givenNames><familyName>Bielack</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au8" creatorRole="author" affiliationRef="#af8"><personName><givenNames>Thomas</givenNames><familyName>Klingebiel</familyName><degrees>MD</degrees></personName></creator><creator xml:id="au9" creatorRole="author" affiliationRef="#af1"><personName><givenNames>Ewa</givenNames><familyName>Koscielniak</familyName><degrees>MD</degrees></personName></creator></creators><affiliationGroup><affiliation xml:id="af1" countryCode="DE" type="organization"><unparsedAffiliation>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Stuttgart, Germany</unparsedAffiliation></affiliation><affiliation xml:id="af2" countryCode="DE" type="organization"><unparsedAffiliation>Institute of Pathology, Heinrich‐Heine‐University, Duesseldorf, Germany</unparsedAffiliation></affiliation><affiliation xml:id="af3" countryCode="DE" type="organization"><unparsedAffiliation>Center of Histopathology, Cytology & Molecular Diagnostics (CHCMD), Research Park Trier, Trier, Germany</unparsedAffiliation></affiliation><affiliation xml:id="af4" countryCode="DE" type="organization"><unparsedAffiliation>Department of Pathology, Institute for Pediatric Pathology, Kiel Pediatric Tumor Registry, University of Kiel, Kiel, Germany</unparsedAffiliation></affiliation><affiliation xml:id="af5" countryCode="PL" type="organization"><unparsedAffiliation>Department of Bone Marrow Transplantation, Pediatric Oncology and Hematology, University of Medicine, Wroclaw, Poland</unparsedAffiliation></affiliation><affiliation xml:id="af6" countryCode="SE" type="organization"><unparsedAffiliation>Department of Pediatric Oncology‐Hematology, University Children's Hospital, Lund, Sweden</unparsedAffiliation></affiliation><affiliation xml:id="af7" countryCode="DE" type="organization"><unparsedAffiliation>Department of Pediatric Hematology and Oncology, University Childrens' Hospital Muenster, Muenster, Germany</unparsedAffiliation></affiliation><affiliation xml:id="af8" countryCode="DE" type="organization"><unparsedAffiliation>Department of Pediatric Oncology, University of Frankfurt (Main), Frankfurt, Germany</unparsedAffiliation></affiliation></affiliationGroup><keywordGroup xml:lang="en" type="author"><keyword xml:id="kwd1">PAX–FKHR</keyword><keyword xml:id="kwd2">prognosis</keyword><keyword xml:id="kwd3">rhabdomyosarcoma</keyword><keyword xml:id="kwd4">RT‐PCR</keyword><keyword xml:id="kwd5">translocation</keyword></keywordGroup><fundingInfo><fundingAgency>Foerderkreis Krebskranke Kinder e.V., Stuttgart, Germany</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Olgaele Stiftung für das kranke Kind e.V., Stuttgart, Germany</fundingAgency></fundingInfo><fundingInfo><fundingAgency>Deutsche Krebshilfe e.V., Bonn, Germany</fundingAgency><fundingNumber>50‐2721</fundingNumber></fundingInfo><supportingInformation><p> Additional Supporting Information may be found in the online version of this article. </p><supportingInfoItem><mediaResource alt="supporting information" href="urn-x:wiley:15455009:media:pbc22958:pbc_22958_sm_SuppTab1"></mediaResource><caption>Supplementary Table 1</caption></supportingInfoItem><supportingInfoItem><mediaResource alt="supporting information" href="urn-x:wiley:15455009:media:pbc22958:pbc_22958_sm_SuppTab2"></mediaResource><caption>Supplementary Table 2</caption></supportingInfoItem><supportingInfoItem><mediaResource alt="supporting information" href="urn-x:wiley:15455009:media:pbc22958:pbc_22958_sm_SuppApp1"></mediaResource><caption>Supplementary Appendix 1</caption></supportingInfoItem><supportingInfoItem><mediaResource alt="supporting information" href="urn-x:wiley:15455009:media:pbc22958:pbc_22958_sm_SuppApp2"></mediaResource><caption>Supplementary Appendix 2</caption></supportingInfoItem></supportingInformation><abstractGroup><abstract type="main" xml:lang="en"><title type="main">Abstract</title><section xml:id="abs1-1"><title type="main">Background</title><p>Alveolar Rhabdomyosarcomas (RMA) are characterized by chromosomal translocations, fusing the PAX3 or PAX7 gene with FKHR in about 85%. Previous studies have suggested that the fusion type is associated with prognosis. In order to investigate the predictive value of the PAX–FKHR fusion status on disease outcome of patients with RMA treated in the CWS trials we performed a retrospective analysis.</p></section><section xml:id="abs1-2"><title type="main">Procedure</title><p>Between 1986 and 2004, out of 446 patients with RMA treated in four consecutive CWS trials, tumor samples from 126 patients were available for RT‐PCR analysis. Survival depending on fusion status in context with known clinical risk‐factors was analyzed.</p></section><section xml:id="abs1-3"><title type="main">Results</title><p>Out of 126 samples, 121 had adequate quality for PAX–FKHR fusion status analysis. PAX–FKHR fusions were detected in 101 samples: 60% PAX3–FKHR and 24% PAX7–FKHR fusions, 17% were fusion‐negative. There was no significant difference in survival between patients with PAX3–FKHR versus PAX7–FKHR positive tumors. The fusion transcript negative cohort showed a more favorable outcome than the fusion transcript positive cohort among patients with metastatic disease. From the established clinical risk‐factors none was associated with a significantly higher risk of failure or death in a multivariate analysis.</p></section><section xml:id="abs1-4"><title type="main">Conclusions</title><p>PAX–FKHR fusion type was not a significant predictor for survival in our analysis. More extensive molecular analyses are needed to identify features with prognostic relevance and useful therapeutic impact. Pediatr Blood Cancer 2011; 57: 406–414. © 2011 Wiley‐Liss, Inc.</p></section></abstract></abstractGroup></contentMeta><noteGroup><note xml:id="fn1"><p>Conflict of Interest: Nothing to report.</p></note></noteGroup></header></component></istex:document></istex:metadataXml><mods version="3.6"><titleInfo lang="en"><title>Prognostic value of PAX–FKHR fusion status in alveolar rhabdomyosarcoma: A report from the cooperative soft tissue sarcoma study group (CWS)</title></titleInfo><titleInfo type="abbreviated" lang="en"><title>PAX–FKHR and Prognosis of RMA</title></titleInfo><titleInfo type="alternative" contentType="CDATA" lang="en"><title>Prognostic value of PAX–FKHR fusion status in alveolar rhabdomyosarcoma: A report from the cooperative soft tissue sarcoma study group (CWS)</title></titleInfo><name type="personal"><namePart type="given">Sabine</namePart><namePart type="family">Stegmaier</namePart><namePart type="termsOfAddress">ScD</namePart><affiliation>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Stuttgart, Germany</affiliation><affiliation>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Bismarckstr. 8, 70176 Stuttgart, Germany.===</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Christopher</namePart><namePart type="family">Poremba</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Institute of Pathology, Heinrich‐Heine‐University, Duesseldorf, Germany</affiliation><affiliation>Center of Histopathology, Cytology & Molecular Diagnostics (CHCMD), Research Park Trier, Trier, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Karl‐Ludwig</namePart><namePart type="family">Schaefer</namePart><namePart type="termsOfAddress">ScD</namePart><affiliation>Institute of Pathology, Heinrich‐Heine‐University, Duesseldorf, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Ivo</namePart><namePart type="family">Leuschner</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Pathology, Institute for Pediatric Pathology, Kiel Pediatric Tumor Registry, University of Kiel, Kiel, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Bernarda</namePart><namePart type="family">Kazanowska</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Bone Marrow Transplantation, Pediatric Oncology and Hematology, University of Medicine, Wroclaw, Poland</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Albert N.</namePart><namePart type="family">Békássy</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Pediatric Oncology‐Hematology, University Children's Hospital, Lund, Sweden</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Stefan S.</namePart><namePart type="family">Bielack</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Stuttgart, Germany</affiliation><affiliation>Department of Pediatric Hematology and Oncology, University Childrens' Hospital Muenster, Muenster, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Thomas</namePart><namePart type="family">Klingebiel</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Department of Pediatric Oncology, University of Frankfurt (Main), Frankfurt, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><name type="personal"><namePart type="given">Ewa</namePart><namePart type="family">Koscielniak</namePart><namePart type="termsOfAddress">MD</namePart><affiliation>Olgahospital, Pediatrics 5 (Oncology, Hematology, Immunology), Klinikum Stuttgart, Stuttgart, Germany</affiliation><role><roleTerm type="text">author</roleTerm></role></name><typeOfResource>text</typeOfResource><genre type="article" displayLabel="article"></genre><originInfo><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><place><placeTerm type="text">Hoboken</placeTerm></place><dateIssued encoding="w3cdtf">2011-09</dateIssued><dateCaptured encoding="w3cdtf">2009-12-15</dateCaptured><dateValid encoding="w3cdtf">2010-11-10</dateValid><copyrightDate encoding="w3cdtf">2011</copyrightDate></originInfo><language><languageTerm type="code" authority="rfc3066">en</languageTerm><languageTerm type="code" authority="iso639-2b">eng</languageTerm></language><physicalDescription><internetMediaType>text/html</internetMediaType><extent unit="figures">2</extent><extent unit="tables">3</extent><extent unit="references">36</extent><extent unit="words">6401</extent></physicalDescription><abstract>Alveolar Rhabdomyosarcomas (RMA) are characterized by chromosomal translocations, fusing the PAX3 or PAX7 gene with FKHR in about 85%. Previous studies have suggested that the fusion type is associated with prognosis. In order to investigate the predictive value of the PAX–FKHR fusion status on disease outcome of patients with RMA treated in the CWS trials we performed a retrospective analysis.</abstract><abstract>Between 1986 and 2004, out of 446 patients with RMA treated in four consecutive CWS trials, tumor samples from 126 patients were available for RT‐PCR analysis. Survival depending on fusion status in context with known clinical risk‐factors was analyzed.</abstract><abstract>Out of 126 samples, 121 had adequate quality for PAX–FKHR fusion status analysis. PAX–FKHR fusions were detected in 101 samples: 60% PAX3–FKHR and 24% PAX7–FKHR fusions, 17% were fusion‐negative. There was no significant difference in survival between patients with PAX3–FKHR versus PAX7–FKHR positive tumors. The fusion transcript negative cohort showed a more favorable outcome than the fusion transcript positive cohort among patients with metastatic disease. From the established clinical risk‐factors none was associated with a significantly higher risk of failure or death in a multivariate analysis.</abstract><abstract>PAX–FKHR fusion type was not a significant predictor for survival in our analysis. More extensive molecular analyses are needed to identify features with prognostic relevance and useful therapeutic impact. Pediatr Blood Cancer 2011; 57: 406–414. © 2011 Wiley‐Liss, Inc.</abstract><note type="content">*Conflict of Interest: Nothing to report.</note><note type="funding">Foerderkreis Krebskranke Kinder e.V., Stuttgart, Germany</note><note type="funding">Olgaele Stiftung für das kranke Kind e.V., Stuttgart, Germany</note><note type="funding">Deutsche Krebshilfe e.V., Bonn, Germany - No. 50‐2721; </note><subject lang="en"><genre>keywords</genre><topic>PAX–FKHR</topic><topic>prognosis</topic><topic>rhabdomyosarcoma</topic><topic>RT‐PCR</topic><topic>translocation</topic></subject><relatedItem type="host"><titleInfo><title>Pediatric Blood & Cancer</title></titleInfo><titleInfo type="abbreviated"><title>Pediatr. Blood Cancer</title></titleInfo><genre type="journal">journal</genre><note type="content"> Additional Supporting Information may be found in the online version of this article.Supporting Info Item: Supplementary Table 1 - Supplementary Table 2 - Supplementary Appendix 1 - Supplementary Appendix 2 - </note><subject><genre>article-category</genre><topic>Research Article</topic></subject><identifier type="ISSN">1545-5009</identifier><identifier type="eISSN">1545-5017</identifier><identifier type="DOI">10.1002/(ISSN)1545-5017</identifier><identifier type="PublisherID">PBC</identifier><part><date>2011</date><detail type="volume"><caption>vol.</caption><number>57</number></detail><detail type="issue"><caption>no.</caption><number>3</number></detail><extent unit="pages"><start>406</start><end>414</end><total>9</total></extent></part></relatedItem><relatedItem type="preceding"><titleInfo><title>Medical and Pediatric Oncology</title></titleInfo><identifier type="ISSN">0096-0802</identifier><identifier type="ISSN">1096-911X</identifier><part><date point="end">2003</date><detail type="volume"><caption>last vol.</caption><number>41</number></detail><detail type="issue"><caption>last no.</caption><number>6</number></detail></part></relatedItem><identifier type="istex">3F2A4A97541A975486E92F1C020E9BEE07E6F707</identifier><identifier type="DOI">10.1002/pbc.22958</identifier><identifier type="ArticleID">PBC22958</identifier><accessCondition type="use and reproduction" contentType="copyright">Copyright © 2011 Wiley‐Liss, Inc.</accessCondition><recordInfo><recordContentSource>WILEY</recordContentSource><recordOrigin>Wiley Subscription Services, Inc., A Wiley Company</recordOrigin></recordInfo></mods></metadata><serie></serie></istex></record>Pour manipuler ce document sous Unix (Dilib)
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