Prognostic Value of PAX-FKHR Fusion Status in Alveolar Rhabdomyosarcoma: A Report From the Cooperative Soft Tissue Sarcoma Study Group (CWS)

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@article{09352edb13f34abeb55dcf0ab6af95e3,
title = "Prognostic Value of PAX-FKHR Fusion Status in Alveolar Rhabdomyosarcoma: A Report From the Cooperative Soft Tissue Sarcoma Study Group (CWS)",
abstract = "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.",
keywords = "PAX-FKHR, prognosis, rhabdomyosarcoma, RT-PCR, translocation",
author = "Sabine Stegmaier and Christopher Poremba and Karl-Ludwig Schaefer and Ivo Leuschner and Bernarda Kazanowska and Albert B{\'e}k{\'a}ssy and Bielack, {Stefan S.} and Thomas Klingebiel and Ewa Koscielniak",
year = "2011",
doi = "10.1002/pbc.22958",
language = "English",
volume = "57",
pages = "406--414",
journal = "Pediatric Blood & Cancer",
issn = "1545-5017",
publisher = "John Wiley and Sons Inc.",
number = "3",

}