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Histopathological patterns in atypical teratoid/rhabdoid tumors are related to molecular subgroup

Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant tumor that may not only contain rhabdoid tumor cells but also poorly differentiated small‐round‐blue cells as well as areas with mesenchymal or epithelial differentiation. Little is known on factors associated with histopathological dive...

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Published in:Brain pathology (Zurich, Switzerland) Switzerland), 2021-09, Vol.31 (5), p.e12967-n/a
Main Authors: Zin, Francesca, Cotter, Jennifer A., Haberler, Christine, Dottermusch, Matthias, Neumann, Julia, Schüller, Ulrich, Schweizer, Leonille, Thomas, Christian, Nemes, Karolina, Johann, Pascal D., Kool, Marcel, Frühwald, Michael C., Paulus, Werner, Judkins, Alexander, Hasselblatt, Martin
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Language:English
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Summary:Atypical teratoid/rhabdoid tumor (AT/RT) is a highly malignant tumor that may not only contain rhabdoid tumor cells but also poorly differentiated small‐round‐blue cells as well as areas with mesenchymal or epithelial differentiation. Little is known on factors associated with histopathological diversity. Recent studies demonstrated three molecular subgroups of AT/RT, namely ATRT‐TYR, ATRT‐SHH, and ATRT‐MYC. We thus aimed to investigate if morphological patterns might be related to molecular subgroup status. Hematoxylin‐eosin stained sections of 114 AT/RT with known molecular subgroup status were digitalized and independently categorized by nine blinded observers into four morphological categories, that is, “rhabdoid,” “small‐round‐blue,” “epithelial,” and “mesenchymal.” The series comprised 48 ATRT‐SHH, 40 ATRT‐TYR, and 26 ATRT‐MYC tumors. Inter‐observer agreement was moderate but significant (Fleiss’ kappa = 0.47; 95% C.I. 0.41‐0.53; p 
ISSN:1015-6305
1750-3639
DOI:10.1111/bpa.12967