Claudin-6 is of limited sensitivity and specificity for the diagnosis of atypical teratoid/rhabdoid tumors.
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.
Department of Radiology, Oncology and Anatomic-pathology Sciences, Sapienza University, Rome, Italy.
Manila
M
Antonelli
Martin
M
Hasselblatt
Christine
C
Haberler
Angela
A
Di Giannatale
Maria L
ML
Garrè
Vittoria
V
Donofrio
Libero
L
Lauriola
Vita
V
Ridola
Antonella
A
Arcella
Michael
M
Frühwald
Felice
F
Giangaspero
D001317:Austria; D001932:Brain Neoplasms / Q000145:classification / Q000175:diagnosis* / Q000378:metabolism* / Q000401:mortality; D016009:Chi-Square Distribution; D002868:Chromosomal Proteins, Non-Histone / Q000378:metabolism; D057167:Claudins / Q000378:metabolism*; D004268:DNA-Binding Proteins / Q000378:metabolism; D005260:Female; D005500:Follow-Up Studies; D005858:Germany; D006801:Humans; D053208:Kaplan-Meier Estimate; D008297:Male; D010372:Pediatrics; D018335:Rhabdoid Tumor / Q000145:classification / Q000175:diagnosis* / Q000378:metabolism* / Q000401:mortality; D000071796:SMARCB1 Protein; D012680:Sensitivity and Specificity; D014157:Transcription Factors / Q000378:metabolism
D002868:Chromosomal Proteins, Non-Histone; D057167:Claudins; D004268:DNA-Binding Proteins; D000071796:SMARCB1 Protein; C513266:SMARCB1 protein, human; D014157:Transcription Factors; C122101:claudin 6
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