Changes in FXR1 Expression After Chemotherapy for Rhabdomyos
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Rhabdomyosarcoma (RMS) arises from abnormal muscle development. Researchers reported previously that Fragile-X-Related 1 ( FXR1), essential to normal myogenesis, was highly expressed in RMS relative to other embryonal tumors. This study suggests that reduced expression of the FXR1 gene is linked with improved survival.

RMS patients lesser than 18 years were categorized according to tumor histology, PAX/FOXO1 translocation, and vital status. FXR1 protein expression was compared before and after chemotherapy. The impact of FXR1 expression on relapse-free (RFS) and overall survival (OS) was analyzed.

--FXR1 was most intensely expressed in the cytosol of undifferentiated rhabdomyoblasts. At diagnosis, FXR1 expression was ubiquitous and strong across all disease characteristics and foremost associated with worse RFS in translocation-positive patients.

--Among embryonal and translocation-negative RMS, survivors showed a significantly greater decrease in FXR1 expression after chemotherapy compared to decedents.

--In contrast, alveolar and translocation-positive RMS specimens showed insignificant changes in FXR1 expression across therapy.

--As expected, alveolar histology, translocation presence, stage, and the clinical group associated with worse survival.

Conclusively, FXR1 was expressed strongly across RMS specimens at diagnosis regardless of disease or patient characteristics, and particularly in undifferentiated cells. Reduction in FXR1 expression after chemotherapy associated with improved survival for embryonal and translocation-negative RMS patients.

Journal of Pediatric Surgery