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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

http://www.scopus.com/inward/record.url?partnerID=HzOxMe3b&scp=84940739507&origin=inward; http://dx.doi.org/10.1371/journal.pone.0133019; http://www.ncbi.nlm.nih.gov/pubmed/26168243; https://dx.plos.org/10.1371/journal.pone.0133019.g002; http://dx.doi.org/10.1371/journal.pone.0133019.g002; https://dx.plos.org/10.1371/journal.pone.0133019.g004; http://dx.doi.org/10.1371/journal.pone.0133019.g004; https://dx.plos.org/10.1371/journal.pone.0133019.t001; http://dx.doi.org/10.1371/journal.pone.0133019.t001; https://dx.plos.org/10.1371/journal.pone.0133019.g005; http://dx.doi.org/10.1371/journal.pone.0133019.g005; https://dx.plos.org/10.1371/journal.pone.0133019; https://dx.plos.org/10.1371/journal.pone.0133019.g001; http://dx.doi.org/10.1371/journal.pone.0133019.g001; https://dx.plos.org/10.1371/journal.pone.0133019.g003; http://dx.doi.org/10.1371/journal.pone.0133019.g003; https://dx.plos.org/10.1371/journal.pone.0133019.g006; http://dx.doi.org/10.1371/journal.pone.0133019.g006; https://dx.doi.org/10.1371/journal.pone.0133019.g004; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0133019.g004; https://dx.doi.org/10.1371/journal.pone.0133019.g002; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0133019.g002; https://dx.doi.org/10.1371/journal.pone.0133019.g003; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0133019.g003; https://dx.doi.org/10.1371/journal.pone.0133019.g005; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0133019.g005; https://dx.doi.org/10.1371/journal.pone.0133019.g006; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0133019.g006; https://dx.doi.org/10.1371/journal.pone.0133019.g001; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0133019.g001; https://dx.doi.org/10.1371/journal.pone.0133019.t001; https://journals.plos.org/plosone/article/figure?id=10.1371/journal.pone.0133019.t001; https://dx.doi.org/10.1371/journal.pone.0133019; https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0133019; http://dx.plos.org/10.1371/journal.pone.0133019.g006; http://dx.plos.org/10.1371/journal.pone.0133019.g003; http://dx.plos.org/10.1371/journal.pone.0133019.g002; http://dx.plos.org/10.1371/journal.pone.0133019; https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0133019&type=printable; http://dx.plos.org/10.1371/journal.pone.0133019.g001; http://www.plosone.org/article/metrics/info:doi/10.1371/journal.pone.0133019; http://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0133019&type=printable; http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0133019; http://dx.plos.org/10.1371/journal.pone.0133019.g005; http://dx.plos.org/10.1371/journal.pone.0133019.t001; http://journals.plos.org/plosone/article/metrics?id=10.1371/journal.pone.0133019; http://dx.plos.org/10.1371/journal.pone.0133019.g004; http://journals.plos.org/plosone/article?id=10.1371%2Fjournal.pone.0133019

Calogero Virgone; Enzo Lalli; Gianni Bisogno; Elena Lazzari; Josep Roma; Angelica Zin; Elena Poli; Giovanni Cecchetto; Patrizia Dall’Igna; Rita Alaggio; Jorge Sans Burns

Public Library of Science (PLoS)

Biochemistry, Genetics and Molecular Biology; Agricultural and Biological Sciences; Multidisciplinary

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