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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Prognostic Value of PAX-FKHR Fusion Status in Alveolar Rhabdomyosarcoma: A Report From the Cooperative Soft Tissue Sarcoma Study Group (CWS). / Stegmaier, Sabine; Poremba, Christopher; Schaefer, Karl-Ludwig; Leuschner, Ivo; Kazanowska, Bernarda; Békássy, Albert; Bielack, Stefan S.; Klingebiel, Thomas; Koscielniak, Ewa.

In: Pediatric Blood & Cancer, Vol. 57, No. 3, 2011, p. 406-414.

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Harvard

Stegmaier, S, Poremba, C, Schaefer, K-L, Leuschner, I, Kazanowska, B, Békássy, A, Bielack, SS, Klingebiel, T & Koscielniak, E 2011, 'Prognostic Value of PAX-FKHR Fusion Status in Alveolar Rhabdomyosarcoma: A Report From the Cooperative Soft Tissue Sarcoma Study Group (CWS)', Pediatric Blood & Cancer, vol. 57, no. 3, pp. 406-414. https://doi.org/10.1002/pbc.22958

APA

Stegmaier, S., Poremba, C., Schaefer, K-L., Leuschner, I., Kazanowska, B., Békássy, A., ... Koscielniak, E. (2011). Prognostic Value of PAX-FKHR Fusion Status in Alveolar Rhabdomyosarcoma: A Report From the Cooperative Soft Tissue Sarcoma Study Group (CWS). Pediatric Blood & Cancer, 57(3), 406-414. https://doi.org/10.1002/pbc.22958

CBE

Stegmaier S, Poremba C, Schaefer K-L, Leuschner I, Kazanowska B, Békássy A, Bielack SS, Klingebiel T, Koscielniak E. 2011. Prognostic Value of PAX-FKHR Fusion Status in Alveolar Rhabdomyosarcoma: A Report From the Cooperative Soft Tissue Sarcoma Study Group (CWS). Pediatric Blood & Cancer. 57(3):406-414. https://doi.org/10.1002/pbc.22958

MLA

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Author

Stegmaier, Sabine ; Poremba, Christopher ; Schaefer, Karl-Ludwig ; Leuschner, Ivo ; Kazanowska, Bernarda ; Békássy, Albert ; Bielack, Stefan S. ; Klingebiel, Thomas ; Koscielniak, Ewa. / Prognostic Value of PAX-FKHR Fusion Status in Alveolar Rhabdomyosarcoma: A Report From the Cooperative Soft Tissue Sarcoma Study Group (CWS). In: Pediatric Blood & Cancer. 2011 ; Vol. 57, No. 3. pp. 406-414.

RIS

TY - JOUR

T1 - Prognostic Value of PAX-FKHR Fusion Status in Alveolar Rhabdomyosarcoma: A Report From the Cooperative Soft Tissue Sarcoma Study Group (CWS)

AU - Stegmaier, Sabine

AU - Poremba, Christopher

AU - Schaefer, Karl-Ludwig

AU - Leuschner, Ivo

AU - Kazanowska, Bernarda

AU - Békássy, Albert

AU - Bielack, Stefan S.

AU - Klingebiel, Thomas

AU - Koscielniak, Ewa

PY - 2011

Y1 - 2011

N2 - 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.

AB - 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.

KW - PAX-FKHR

KW - prognosis

KW - rhabdomyosarcoma

KW - RT-PCR

KW - translocation

U2 - 10.1002/pbc.22958

DO - 10.1002/pbc.22958

M3 - Article

VL - 57

SP - 406

EP - 414

JO - Pediatric Blood & Cancer

JF - Pediatric Blood & Cancer

SN - 1545-5017

IS - 3

ER -