{"doi":"10.1093/neuonc/noab247","title":"Prevalence of <i>BRAF</i>V600 in glioma and use of <i>BRAF</i> Inhibitors in patients with <i>BRAF</i>V600 mutation-positive glioma: systematic review","abstract":"<jats:title>Abstract</jats:title>\n               <jats:sec>\n                  <jats:title>Background</jats:title>\n                  <jats:p>Detailed prevalence estimates of BRAFV600 mutations and BRAF inhibitor (BRAFi) treatment responses in V600-mutant glioma will inform trial development.</jats:p>\n               </jats:sec>\n               <jats:sec>\n                  <jats:title>Methods</jats:title>\n                  <jats:p>Our systematic review analyzed overall prevalence of BRAFV600 mutations in glioma and BRAFi treatment response.</jats:p>\n               </jats:sec>\n               <jats:sec>\n                  <jats:title>Results</jats:title>\n                  <jats:p>Based on 13 682 patients in 182 publications, the prevalence of BRAFV600 in epithelioid glioblastoma (eGBM) was 69% [95% CI: 45–89%]; pleomorphic xanthoastrocytoma (PXA): 56% [48–64%] anaplastic pleomorphic xanthoastrocytoma (aPXA): 38% [23–54%], ganglioglioma (GG): 40% [33–46%], and anaplastic ganglioglioma (aGG): 46% [18–76%]. Prevalence in astroblastoma was 24% [8–43%], desmoplastic infantile astrocytoma (DIA): 16% [0–57%], subependymal giant cell astrocytoma (SEGA): 8% [0–37%], dysembryoplastic neuroepithelial tumor (DNET): 3% [0–11%], diffuse astrocytoma (DA): 3% [0–9%], and pilocytic astrocytoma (PA): 3% [2–5%]. We reviewed 394 V600-mutant gliomas treated with BRAFi from 130 publications. One hundred and twenty-nine pediatric low-grade gliomas showed 4 (3.1%) complete response (CR); 53 (41.1%) partial response (PR); 64 (49.6%) stable disease (SD) and 8 (6.2%) progressive disease (PD). 25 pediatric high-grade gliomas showed CR; PR; SD; PD in 4 (16.0%); 10 (40.0%), 4 (16.0%); and 7 (28.0%) respectively. Thirty-nine adult low-grade gliomas showed CR; PR; SD; PD of 4 (10.3%); 17 (43.6%); 16 (41.0%) and 2 (5.1%) respectively. Ninety-seven adult high-grade gliomas showed CR; PR; SD; PD of 6 (6.2%); 31 (32.0%); 27 (27.8%); and 33 (34.0%) respectively.</jats:p>\n               </jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions</jats:title>\n                  <jats:p>BRAFV600 prevalence is highest in eGBM, PXA, aPXA, GG, aGG, and lower in astroblastoma, DIA, SEGA, DNET, DA, and PA. Our data provide the rationale for adjuvant clinical trials of BRAFi in V600-mutant glioma.</jats:p>\n               </jats:sec>","journal":"Neuro-Oncology","year":2022,"id":634967,"datarank":0.62147020895873,"base_score":4.143134726391533,"endowment":4.143134726391533,"self_citation_contribution":0.62147020895873,"citation_network_contribution":0.0,"self_endowment_contribution":0.62147020895873,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":62,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1647099,"name":"Zak A Thornton","orcid":"0000-0001-9275-7198","position":1,"is_corresponding":false},{"id":1647100,"name":"Saanwalshah S Saincher","orcid":"0000-0002-5865-9874","position":2,"is_corresponding":false},{"id":1647101,"name":"Ian Y Yao","orcid":null,"position":3,"is_corresponding":false},{"id":272156,"name":"Sarah Dawson","orcid":"0000-0002-6682-063X","position":4,"is_corresponding":false},{"id":16509,"name":"Luke A. McGuinness","orcid":"0000-0001-8730-9761","position":5,"is_corresponding":false},{"id":3376,"name":"Hayley E. Jones","orcid":"0000-0002-4265-2854","position":6,"is_corresponding":false},{"id":1647102,"name":"Sarah Jefferies","orcid":null,"position":7,"is_corresponding":false},{"id":1029255,"name":"Susan Short","orcid":"0000-0003-4423-7256","position":8,"is_corresponding":false},{"id":89501,"name":"Hung‐Yuan Cheng","orcid":"0000-0002-6162-4146","position":9,"is_corresponding":false},{"id":1198702,"name":"Alexandra McAleenan","orcid":"0000-0003-2119-5604","position":10,"is_corresponding":false},{"id":1502,"name":"Julian P. T. Higgins","orcid":"0000-0002-8323-2514","position":11,"is_corresponding":false},{"id":236589,"name":"Kathreena M. Kurian","orcid":"0000-0003-0303-3716","position":12,"is_corresponding":false},{"id":1647098,"name":"Lily J Andrews","orcid":"0000-0001-5595-1987","position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"Prevalence of <i>BRAF</i>V600 in glioma and use of <i>BRAF</i> Inhibitors in patients with <i>BRAF</i>V600 mutation-positive glioma: systematic review","abstract":"<jats:title>Abstract</jats:title>\n               <jats:sec>\n                  <jats:title>Background</jats:title>\n                  <jats:p>Detailed prevalence estimates of BRAFV600 mutations and BRAF inhibitor (BRAFi) treatment responses in V600-mutant glioma will inform trial development.</jats:p>\n               </jats:sec>\n               <jats:sec>\n                  <jats:title>Methods</jats:title>\n                  <jats:p>Our systematic review analyzed overall prevalence of BRAFV600 mutations in glioma and BRAFi treatment response.</jats:p>\n               </jats:sec>\n               <jats:sec>\n                  <jats:title>Results</jats:title>\n                  <jats:p>Based on 13 682 patients in 182 publications, the prevalence of BRAFV600 in epithelioid glioblastoma (eGBM) was 69% [95% CI: 45–89%]; pleomorphic xanthoastrocytoma (PXA): 56% [48–64%] anaplastic pleomorphic xanthoastrocytoma (aPXA): 38% [23–54%], ganglioglioma (GG): 40% [33–46%], and anaplastic ganglioglioma (aGG): 46% [18–76%]. Prevalence in astroblastoma was 24% [8–43%], desmoplastic infantile astrocytoma (DIA): 16% [0–57%], subependymal giant cell astrocytoma (SEGA): 8% [0–37%], dysembryoplastic neuroepithelial tumor (DNET): 3% [0–11%], diffuse astrocytoma (DA): 3% [0–9%], and pilocytic astrocytoma (PA): 3% [2–5%]. We reviewed 394 V600-mutant gliomas treated with BRAFi from 130 publications. One hundred and twenty-nine pediatric low-grade gliomas showed 4 (3.1%) complete response (CR); 53 (41.1%) partial response (PR); 64 (49.6%) stable disease (SD) and 8 (6.2%) progressive disease (PD). 25 pediatric high-grade gliomas showed CR; PR; SD; PD in 4 (16.0%); 10 (40.0%), 4 (16.0%); and 7 (28.0%) respectively. Thirty-nine adult low-grade gliomas showed CR; PR; SD; PD of 4 (10.3%); 17 (43.6%); 16 (41.0%) and 2 (5.1%) respectively. Ninety-seven adult high-grade gliomas showed CR; PR; SD; PD of 6 (6.2%); 31 (32.0%); 27 (27.8%); and 33 (34.0%) respectively.</jats:p>\n               </jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions</jats:title>\n                  <jats:p>BRAFV600 prevalence is highest in eGBM, PXA, aPXA, GG, aGG, and lower in astroblastoma, DIA, SEGA, DNET, DA, and PA. Our data provide the rationale for adjuvant clinical trials of BRAFi in V600-mutant glioma.</jats:p>\n               </jats:sec>","is_dataset_classified":null,"base_score":4.143134726391533,"endowment":4.143134726391533,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"34718782","pmcid":"PMC8972326","openalex_id":"https://openalex.org/W3211316240","authors":[],"funders":[{"funder_name":"Cancer Research UK","grant_id":"C30758/A29791","title":null},{"funder_name":"Cancer Research UK","grant_id":"C18281/A29019","title":null},{"funder_name":"Doctoral Research Fellowship","grant_id":"DRF-2018-11-ST2-048","title":null},{"funder_name":"National Institute for Health Research","grant_id":"NF-SI-0617-10145","title":null},{"funder_name":"Medical Research Council","grant_id":"G1100578","title":null},{"funder_name":"Medical Research Council","grant_id":"MR/N004272/1","title":null},{"funder_name":"Medical Research Council","grant_id":"G0701018","title":null},{"funder_name":"Medical Research Council","grant_id":"MR/M014533/1","title":null},{"funder_name":"Medical Research Council","grant_id":"MR/T044594/1","title":"HCD: Synthesis of networks of evidence on test accuracy, with and without a 'gold standard'"},{"funder_name":"Cancer Research UK","grant_id":"29019","title":null},{"funder_name":"Cancer Research UK","grant_id":"S_4344","title":null},{"funder_name":"Bristol Biomedical Research Centre at University Hospitals Bristol","grant_id":"","title":null},{"funder_name":"University of Bristol","grant_id":"","title":null},{"funder_name":"Weston NHS Foundation Trust","grant_id":"","title":null},{"funder_name":"Southmead Hospital Charitable Funds: Brain Tumour Bank","grant_id":"","title":null}],"total_grants":15,"fwci":3.7155,"citation_percentile":0.95581395,"influential_citations":0,"citation_trend":[{"year":2022,"count":8},{"year":2023,"count":16},{"year":2024,"count":18},{"year":2025,"count":13},{"year":2026,"count":6}],"oa_status":"hybrid","license":"cc-by-nc","oa_locations":[{"url":"https://academic.oup.com/neuro-oncology/article-pdf/24/4/528/43218934/noab247.pdf","host_type":"journal"},{"url":"https://academic.oup.com/neuro-oncology/article-pdf/24/4/528/43218934/noab247.pdf","host_type":"publisher"},{"url":"https://academic.oup.com/neuro-oncology/advance-article-pdf/doi/10.1093/neuonc/noab247/41685914/noab247.pdf","host_type":"publisher"},{"url":"https://doi.org/10.1093/neuonc/noab247","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/34718782","host_type":"repository"},{"url":"https://research-information.bris.ac.uk/en/publications/ec86bccc-ee6f-49a6-993e-fffeee810d95","host_type":"repository"},{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/8972326","host_type":"repository"},{"url":"https://europepmc.org/articles/PMC8972326","host_type":"Europe_PMC"},{"url":"https://europepmc.org/articles/PMC8972326?pdf=render","host_type":"Europe_PMC"},{"url":"http://dx.doi.org/10.1093/neuonc/noab247","host_type":""},{"url":"https://dx.doi.org/10.1093/neuonc/noab247","host_type":""},{"url":"https://hdl.handle.net/1983/ec86bccc-ee6f-49a6-993e-fffeee810d95","host_type":""},{"url":"https://research-information.bris.ac.uk/ws/files/300674561/noab247.pdf","host_type":""},{"url":"https://doi.org/https://doi.org/10.1093/neuonc/noab247","host_type":""}],"fields_of_study":["Glioma Diagnosis and Treatment","Neurofibromatosis and Schwannoma Cases","Neuroblastoma Research and Treatments","03 medical and health sciences","0302 clinical medicine","Adult","Astrocytoma","Brain Neoplasms","Child","Glioma","Humans","Mutation","Prevalence","Proto-Oncogene Proteins B-raf"],"mesh_terms":["Adult","Astrocytoma","Brain Neoplasms","Child","Glioma","Humans","Mutation","Prevalence","Proto-Oncogene Proteins B-raf"],"keywords":["Glioma","Medicine","Mutation","Mutant","Oncology","Cancer research","Internal medicine","Biology","Genetics","Gene","Prevalence","Systematic review","Braf","Braf Inhibitors","Adult","Proto-Oncogene Proteins B-raf","Brain Neoplasms","name=ICEP","610","600","Astrocytoma","/dk/atira/pure/core/keywords/icep","Metadata Analysis/Review","Humans","Child","/dk/atira/pure/core/keywords/icep; name=ICEP"],"sdg_mappings":[{"sdg_number":3,"sdg_label":"3. Good health"},{"sdg_number":0,"sdg_label":"Good health and well-being"}],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[{"name":"nct"}],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-06T14:26:34.831005Z","pmid":null,"pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}