DAX-1 expression in pediatric rhabdomyosarcomas: Another immunohistochemical marker useful in the diagnosis of translocation positive alveolar rhabdomyosarcoma
PLoS ONE, ISSN: 1932-6203, Vol: 10, Issue: 7, Page: e0133019
2015
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Article Description
Objectives: The aim of this study was to investigate the expression of DAX-1 in a series of pediatric rhabdomyosarcomas (RMS) with known translocation and compare it to Ap2β, known to be selectively expressed in ARMS. Design: We revised a series of 71 alveolar rhabdomyosarcomas (ARMS), enrolled in the Italian Protocols RMS 79 and 96, and 23 embryonal rhabdomyosarcomas (ERMS) as controls. Before investigating Ap2β and DAX-1, ARMS were reviewed and reclassified as 48 ARMS and 23 non-ARMS. Results: Translocation positive ARMS showed a characteristic Ap2β/DAX-1+ staining pattern in 78% of cases, while 76% of classic ERMS were negative for both. Ap2β alone was positive in 3.9% of RMS lacking translocation, whereas DAX-1 alone was positive in 25.4%. Conversely, 9% and 6% of translocation positive ARMS were positive only for DAX-1 or Ap2β, respectively. The 23 non-ARMS shared the same phenotype as ERMS but had a higher frequency of DAX-1 expression. Conclusions: DAX-1 is less specific than Ap2β, however it is a sensitive marker for translocation positive ARMS and can be helpful in their diagnosis if used in combination with Ap2β.
Bibliographic Details
10.1371/journal.pone.0133019; 10.1371/journal.pone.0133019.g002; 10.1371/journal.pone.0133019.g004; 10.1371/journal.pone.0133019.t001; 10.1371/journal.pone.0133019.g005; 10.1371/journal.pone.0133019.g001; 10.1371/journal.pone.0133019.g003; 10.1371/journal.pone.0133019.g006
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