Claudin-6 is of Limited Sensitivity and Specificity for the Diagnosis of Atypical Teratoid/Rhabdoid Tumors.

Massimo Antonelli, M Hasselblatt, C Haberler, Angela Di Giannatale, Ml Garre', V Donofrio, Libero Lauriola, Vita Ridola, A Arcella, M Früwald, Felice Giangaspero

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12 Citazioni (Scopus)


Recent gene expression microarray analyses have indicated that claudin-6 is specifically expressed in atypical teratoid rhabdoid tumors (AT/RTs), suggesting a role as a positive diagnostic marker in addition to SMARCB1 (INI1) loss, which is encountered in the majority of AT/RTs. In order to investigate the potential of claudin-6 as a diagnostic marker, expression was investigated in 59 AT/RTs and 60 other primary central nervous system (CNS) tumors, including primitive neuroectodermal tumors, medulloblastomas, choroid plexus tumors, and both pediatric and adult low- and high-grade gliomas using immunohistochemistry. Claudin-6 was expressed in 17/59 AT/RTs (29%), but also in a variety of other primary CNS tumors, including 60% of medulloblastomas and 21% of malignant gliomas. Even though high staining scores (2+ or 3+) were more often encountered in AT/RTs (Chi-square 4.177; P = 0.041), the overall frequency of claudin-6 staining was not significantly higher in AT/RTs as compared with the other tumors (17/59 vs. 16/60; Chi-square = 0.328; P = 0.567). In a subgroup of 43 AT/RT patients, of which follow-up data were available, claudin-6 expression did not show any correlation with survival. In conclusion, claudin-6 immunohistochemistry is of limited sensitivity and specificity for the diagnosis of AT/RT and does not correlate with clinical behavior.
Lingua originaleEnglish
pagine (da-a)558-563
Numero di pagine6
RivistaBrain Pathology
Stato di pubblicazionePubblicato - 2011


  • rhabdoid
  • teratoid


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