PAX3/FOXO1 Fusion Gene Status Key to Managing Rhabdomyosarcoma Risk

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(ChemotherapyAdvisor) – A continuous risk score derived from combining clinical parameters with presence or absence of the PAX3/FOXO1 fusion gene “represents a robust approach to improving current risk-adapted therapy for rhabdomyosarcoma,” investigators concluded in the Journal of Clinical Oncology online March 26.

“This study offers a blueprint for how molecular data can be applied to the management of rhabdomyosarcoma risk,” they wrote. The multinational trial conducted a meta-analysis on two independent data sets of gene-expression profiling for 124 and 101 patients with rhabdomyosarcoma to derive prognostic gene signatures. These signatures, along with one metagene signature previously published, were evaluated using cross-validation analyses.

A combined clinical and molecular risk-stratification scheme incorporating PAX3/FOXO1 fusion gene status was derived from 287 patients with rhabdomyosarcoma and evaluated. All three signatures performed well, with reproducible and significant effects; however, this effect was reduced when cross validated or tested in independent data and did not add new prognostic information over the fusion gene status, which is simpler to assay,” they noted.

Among those without metastases, PAX3/FOXO1 positive patients had a significantly poorer outcome vs. both alveolar-negative and PAX7/FOXO1-positive patients. “Furthermore, a new clinicomolecular risk score that incorporated fusion gene status (negative and PAX3/FOXO1 and PAX7/FOXO1 positive), Intergroup Rhabdomyosarcoma Study TNM stage, and age showed a significant increase in performance over the current risk-stratification scheme.”


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