ArticleBrain pathology (Zurich, Switzerland)2017
Cribriform neuroepithelial tumor: molecular characterization of a SMARCB1-deficient non-rhabdoid tumor with favorable long-term outcome.
Article in Brain pathology (Zurich, Switzerland), 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 32 papers, 2 of them syntheses that pooled it.
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Who cites it
32 citing papers in PubMed, 2 syntheses or guidelines pooled it, 76 citations in OpenAlex.
- Molecular subgrouping of atypical teratoid/rhabdoid tumors-a reinvestigation and current consensus.Neuro-oncology · 2020Pooled it
- Germline variants in SMARCB1 and other members of the BAF chromatin-remodeling complex across human disease entities: a meta-analysis.European journal of human genetics : EJHG · 2018Pooled it
- Aggressive atypical teratoid/rhabdoid tumor with extensive leptomeningeal metastasis in an 8-year-old boy: a case report and narrative review.Translational pediatrics · 2026Article
- SMARCB1-related schwannomatosis and other SMARCB1-associated phenotypes: clinical spectrum and molecular pathogenesis.Familial cancer · 2025Review
- Molecular Testing for the World Health Organization Classification of Central Nervous System Tumors: A Review.JAMA oncology · 2025Review
- Late-onset tumors in rhabdoid tumor predisposition syndrome type-1 (RTPS1) and implications for surveillance.European journal of human genetics : EJHG · 2024Article
- Targeting cholesterol biosynthesis for AT/RT: comprehensive expression analysis and validation in newly established AT/RT cell line.Human cell · 2024Article
- Current status of DNA methylation profiling in neuro-oncology as a diagnostic support tool: A review.Neuro-oncology practice · 2023Review
- Recent insights into the SWI/SNF complex and the molecular mechanism of hSNF5 deficiency in rhabdoid tumors.Cancer medicine · 2023Review
- Transcallosal and endoscopic hybrid approach to a rare entity of pediatric intraventricular tumors-cribriform neuroepithelial tumor: a case report and literature review.Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2023Review
- Epigenetic Regulation in Primary CNS Tumors: An Opportunity to Bridge Old and New WHO Classifications.Cancers · 2023Review
- Newly Recognized CNS Tumors in the 2021 World Health Organization Classification: Imaging Overview with Histopathologic and Genetic Correlation.AJNR. American journal of neuroradiology · 2023Review
- Changes to pediatric brain tumors in 2021 World Health Organization classification of tumors of the central nervous system.Pediatric radiology · 2023Review
- Current Molecular and Clinical Landscape of ATRT - The Link to Future Therapies.Cancer management and research · 2023Review
- Embryonal Tumors of the Central Nervous System with Multilayered Rosettes and Atypical Teratoid/Rhabdoid Tumors.Advances in experimental medicine and biology · 2023Article
- "Adult rhabdoid tumors-a riddle inside an enigma?"Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2022Article
- Article
- 2021 WHO classification of tumours of the central nervous system: a review for the neuroradiologist.Neuroradiology · 2022Review
- Major Features of the 2021 WHO Classification of CNS Tumors.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2022Review
- Clinical implications of the 2021 edition of the WHO classification of central nervous system tumours.Nature reviews. Neurology · 2022Review
Corrections and comments
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Authors and funding
26 authors at 19 institutions in 9 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Rhabdoid phenotype and loss of SMARCB1 expression in a brain tumor are characteristic features of atypical teratoid/rhabdoid tumors (ATRT). Rare non-rhabdoid brain tumors showing cribriform growth pattern and SMARCB1 loss have been designated cribriform neuroepithelial tumor (CRINET). Small case series suggest that CRINETs may have a relatively favorable prognosis. However, the long-term outcome is unclear and it remains uncertain whether CRINET represents a distinct entity or a variant of ATRT. Therefore, 10 CRINETs were clinically and molecularly characterized and compared with 10 ATRTs of each of three recently described molecular subgroups (i.e. ATRT-TYR, ATRT-SHH and ATRT-MYC) using Illumina Infinium HumanMethylation450 arrays, FISH, MLPA, and sequencing. Furthermore, outcome was compared to a larger cohort of 27 children with ATRT-TYR. Median age of the 6 boys and 4 girls harboring a CRINET was 20 months. On histopathological examination, all CRINETs demonstrated a cribriform growth pattern and distinct tyrosinase staining. On unsupervised cluster analysis of methylation data, all CRINETs examined exclusively clustered within the ATRT-TYR molecular subgroup. As ATRT-TYR, CRINETs mainly showed large heterozygous 22q deletions (9/10) and SMARCB1 mutations of the other allele. In two patients, SMARCB1 mutations were also present in the germline. Estimated mean overall survival in patients with CRINETs was 125 months (95% confidence interval 100-151 months) as compared to only 53 (33-74) months in patients with ATRTs of the ATRT-TYR subgroup (Log-Rank P < 0.05). In conclusion, CRINET represents a SMARCB1-deficient non-rhabdoid tumor, which shares molecular similarities with the ATRT-TYR subgroup but has distinct histopathological features and favorable long-term outcome.
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