ArticleNeuro-oncology2017
Germline and somatic BAP1 mutations in high-grade rhabdoid meningiomas.
Article in Neuro-oncology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 120 papers, 2 of them syntheses that pooled it.
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Who cites it
120 citing papers in PubMed, 2 syntheses or guidelines pooled it, 184 citations in OpenAlex.
- Society for Immunotherapy of Cancer (SITC) clinical practice guideline on immunotherapy for the treatment of lung cancer and mesothelioma.Journal for immunotherapy of cancer · 2022Guideline
- EANO guideline on the diagnosis and management of meningiomas.Neuro-oncology · 2021Guideline
- Alliance A071401: Phase II Trial of Focal Adhesion Kinase Inhibition in Meningiomas With SomaticJournal of clinical oncology : official journal of the American Society of Clinical Oncology · 2023Trial
- Clinical and Molecular Features of BAP1-Mutated Meningiomas: Case Series.Molecular carcinogenesis · 2026Article
- Epigenetic Alterations in Meningiomas-A Review.Biomedicines · 2026Review
- Distinct molecular subgroups in pediatric and young-onset meningiomas require age-adapted risk stratification.Nature communications · 2026Article
- The New Tumor Predisposition Syndromes with Neuro-Oncological Relevance-A Comprehensive Review for Neuroradiologists.Clinical neuroradiology · 2026Review
- Meningioma with trabecular architecture: a harbinger of BAP1 deficiency.Brain tumor pathology · 2026Article
- Patient-derived outcome assessment of knowledge, communication, and management in those diagnosed with BAP1-tumor predisposition syndrome.Familial cancer · 2026Article
- High frequency and unique subtypes of meningioma in patients with BAP1 tumor predisposition syndrome.Journal of neuro-oncology · 2026Article
- A comparative evaluation of canine meningioma supporting the canine patient as a naturally occurring animal model for human meningioma: a report from the NCI Comparative Brain Tumor Consortium (CBTC) meningioma pathology board.Veterinary oncology (London, England) · 2026Article
- Advances in molecular and genetic profiling of meningiomas for improved diagnosis, prognosis, and targeted therapy.Frontiers in oncology · 2026Review
- YBX1&YBX3 as novel targets to potentiate immune checkpoint blockade response in gliomas.Neuro-oncology · 2026Article
- High Frequency and Unique Subtypes of Meningioma in Patients with BAP1 Tumor Predisposition Syndrome.medRxiv : the preprint server for health sciences · 2025Article
- First Report of BAP1-Associated Polyposis.American journal of medical genetics. Part A · 2025Article
- Subgroup of meningiomas involving FOS and FOSB gene fusions.Nature communications · 2025Article
- Evolving Treatment Paradigms of Meningiomas; a Precision Medicine Perspective.Current neurology and neuroscience reports · 2025Review
- Molecular signatures define BAP1-altered meningioma as a distinct CNS tumor with deregulation of Polycomb repressive complex target genes.Neuro-oncology · 2025Article
- Germline Non-CDKN2A Variants in Melanoma and Associated Hereditary Cancer Syndromes.Diseases (Basel, Switzerland) · 2025Review
- An overview of BAP1 biological functions and current therapeutics.Biochimica et biophysica acta. Reviews on cancer · 2025Review
60 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
30 authors at 8 institutions in 2 countries.
Funding
Abstract
Background: Patients with meningiomas have widely divergent clinical courses. Some entirely recover following surgery alone, while others have relentless tumor recurrences. This clinical conundrum is exemplified by rhabdoid meningiomas, which are designated in the World Health Organization Classification of Tumours as high grade, despite only a subset following an aggressive clinical course. Patient management decisions are further exacerbated by high rates of interobserver variability, biased against missing possibly aggressive tumors. Objective molecular determinants are needed to guide classification and clinical decision making. Methods: To define genomic aberrations of rhabdoid meningiomas, we performed sequencing of cancer-related genes in 27 meningiomas from 18 patients with rhabdoid features and evaluated breast cancer [BRCA]1-associated protein 1 (BAP1) expression by immunohistochemistry in 336 meningiomas. We assessed outcomes, germline status, and family history in patients with BAP1-negative rhabdoid meningiomas. Results: The tumor suppressor gene BAP1, a ubiquitin carboxy-terminal hydrolase, is inactivated in a subset of high-grade rhabdoid meningiomas. Patients with BAP1-negative rhabdoid meningiomas had reduced time to recurrence compared with patients with BAP1-retained rhabdoid meningiomas (Kaplan-Meier analysis, 26 mo vs 116 mo, P < .001; hazard ratio 12.89). A subset of patients with BAP1-deficient rhabdoid meningiomas harbored germline BAP1 mutations, indicating that rhabdoid meningiomas can be a harbinger of the BAP1 cancer predisposition syndrome. Conclusion: We define a subset of aggressive rhabdoid meningiomas that can be recognized using routine laboratory tests. We implicate ubiquitin deregulation in the pathogenesis of these high-grade malignancies. In addition, we show that familial and sporadic BAP1-mutated rhabdoid meningiomas are clinically aggressive, requiring intensive clinical management.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.