ArticleBrain pathology (Zurich, Switzerland)2021
Histopathological patterns in atypical teratoid/rhabdoid tumors are related to molecular subgroup.
Article in Brain pathology (Zurich, Switzerland), 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers, 1 of them a synthesis that pooled it.
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Who cites it
25 citing papers in PubMed, 1 synthesis or guideline pooled it, 38 citations in OpenAlex.
- Atypical teratoid/rhabdoid tumor in adults: a systematic review of the literature with meta-analysis and additional reports of 4 cases.Journal of neuro-oncology · 2022Pooled it
- A 30-year-old woman with progressive lower extremity sensory loss and weakness.Brain pathology (Zurich, Switzerland) · 2026Article
- Molecular changes during AT/RT progression associated with epithelial-mesenchymal transition and extracellular matrix changes.Acta neuropathologica · 2026Article
- Clinicopathological Characterization of Pediatric Atypical Teratoid/Rhabdoid Tumors and an HE-IHC Dual-Path Deep Learning Model for Auxiliary Diagnosis.Diagnostics (Basel, Switzerland) · 2026Article
- Aggressive atypical teratoid/rhabdoid tumor with extensive leptomeningeal metastasis in an 8-year-old boy: a case report and narrative review.Translational pediatrics · 2026Article
- How is rosette formation in brain tumours linked with cerebrospinal fluid spread?Brain tumor pathology · 2026Review
- Histogenesis of Atypical Teratoid Rhabdoid Tumors: Anatomical and Embryological Perspectives.Cancers · 2025Article
- Role of immunohistochemistry in the molecular classification of atypical teratoid/rhabdoid tumor.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025Article
- Potential clinical value of circulating tumor cells in predicting progression for atypical teratoid rhabdoid tumor in young children.Journal of neuro-oncology · 2025Article
- Unusual Dual Brain Tumor Morphologies in an MEN1 Patient: A Case Report of Diagnostic Challenges and Methylation Insights.International journal of molecular sciences · 2025Article
- Specific imaging features of sellar atypical teratoid/rhabdoid tumor or the lack of thereof.World journal of radiology · 2025Review
- Importance of symptoms acuity for clinical diagnosis of primary sellar atypical teratoid/rhabdoid tumor.World journal of clinical oncology · 2025Review
- Clinical characteristics of patients with atypical teratoid/rhabdoid tumors: a monocentric retrospective analysis.Frontiers in pediatrics · 2025Article
- Autologous stem cell transplantation in adults with atypical teratoid rhabdoid tumor: a case report and review.CNS oncology · 2024Review
- Integrated proteomics spotlight the proteasome as a therapeutic vulnerability in embryonal tumors with multilayered rosettes.Neuro-oncology · 2024Article
- Atypical teratoid rhabdoid tumor in a lower middle‑income country: Challenges to cure.Oncology letters · 2024Article
- Pediatric CNS tumors and 2021 WHO classification: what do oncologists need from pathologists?Frontiers in molecular neuroscience · 2024Review
- Recurrent atypical teratoid/rhabdoid tumors (AT/RT) reveal discrete features of progression on histology, epigenetics, copy number profiling, and transcriptomics.Acta neuropathologica · 2023Article
- Children's Oncology Group's 2023 blueprint for research: Central nervous system tumors.Pediatric blood & cancer · 2023Article
- Pediatric Posterior Fossa ATRT: A Case Report, New Treatment Strategies and Perspectives.Brain sciences · 2023Article
Corrections and comments
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Authors and funding
15 authors at 7 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
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 < 0.001) and there was a highly significant overall association between morphological categories and molecular subgroups for each of the nine observers (p < 0.0001). Specifically, the category "epithelial" was found to be over-represented in ATRT-TYR (p < 0.000001) and the category "small-round-blue" to be over-represented in ATRT-SHH (p < 0.01). The majority of ATRT-MYC was categorized as "mesenchymal" or "rhabdoid," but this association was less compelling. The specificity of the category "epithelial" for ATRT-TYR was highest and accounted for 97% (range: 88-99%) whereas sensitivity was low [49% (range: 35%-63%)]. In line with these findings, cytokeratin-positivity was highly overrepresented in ATRT-TYR. In conclusion, morphological features of AT/RT might reflect molecular alterations and may also provide a first hint on molecular subgroup status, which will need to be confirmed by DNA methylation profiling.
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