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Prognostic Value of...
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Stegmaier, Sabine
(författare)
Prognostic Value of PAX-FKHR Fusion Status in Alveolar Rhabdomyosarcoma: A Report From the Cooperative Soft Tissue Sarcoma Study Group (CWS)
- Artikel/kapitelEngelska2011
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LIBRIS-ID:oai:lup.lub.lu.se:09352edb-13f3-4abe-b55d-cf0ab6af95e3
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https://lup.lub.lu.se/record/2071832URI
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https://doi.org/10.1002/pbc.22958DOI
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Språk:engelska
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Sammanfattning på:engelska
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Ämneskategori:art swepub-publicationtype
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Ämneskategori:ref swepub-contenttype
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Background. 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. Procedure. 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. Results. 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. Conclusions. PAX-FKHR fusion type was not a significant predictor for survival in our analysis. Moreextensive molecular analyses are needed to identify features with prognostic relevance and useful therapeutic impact. Pediatr Blood Cancer 2011; 57: 406-414. (C) 2011 Wiley-Liss, Inc.
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Biuppslag (personer, institutioner, konferenser, titlar ...)
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Poremba, Christopher
(författare)
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Schaefer, Karl-Ludwig
(författare)
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Leuschner, Ivo
(författare)
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Kazanowska, Bernarda
(författare)
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Békássy, AlbertLund University,Lunds universitet,Pediatrik, Lund,Sektion V,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Paediatrics (Lund),Section V,Department of Clinical Sciences, Lund,Faculty of Medicine(Swepub:lu)al4445be
(författare)
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Bielack, Stefan S.
(författare)
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Klingebiel, Thomas
(författare)
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Koscielniak, Ewa
(författare)
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Pediatrik, LundSektion V
(creator_code:org_t)
Sammanhörande titlar
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Ingår i:Pediatric Blood & Cancer: Wiley57:3, s. 406-4141545-50171545-5009
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