{"doi":"10.3389/fonc.2025.1587752","title":"Case Report: Advanced grade 2 meningioma with PBRM1 inactivation with prolonged response to immunotherapy","abstract":"Meningiomas are the most common primary tumor in the central nervous system, yet an effective systemic treatment remains a challenge. We present a grade 2 meningioma that resulted in a positive and prolonged response to pembrolizumab. Our case had polybromo-1 ( PBRM1 ) and BAP1 functional loss, tumor mutational burden of 4 Muts/Mb, stable microsatellite status, and a PD-L1 tumor proportion score of <1%. We add to the limited literature regarding PBRM1 mutations in meningiomas. We discuss our findings in relation to the ongoing investigation of immune checkpoint inhibitor therapy in treating higher-grade refractory meningiomas.","journal":"Frontiers in Oncology","year":2025,"id":578715,"datarank":0.0,"base_score":0.0,"endowment":0.0,"self_citation_contribution":0.0,"citation_network_contribution":0.0,"self_endowment_contribution":0.0,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":0,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9461,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2025-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1488633,"name":"Keng Hee Peh","orcid":"0000-0002-2569-3910","position":1,"is_corresponding":false},{"id":1488634,"name":"Rachael Morgan","orcid":"0000-0001-9589-6388","position":2,"is_corresponding":false},{"id":1488988,"name":"Harry D. Momo","orcid":null,"position":3,"is_corresponding":false},{"id":837719,"name":"David K. Orren","orcid":null,"position":4,"is_corresponding":false},{"id":1488635,"name":"Stephanie Rock","orcid":"0000-0001-5700-6954","position":5,"is_corresponding":false},{"id":1114218,"name":"Thomas Pittman","orcid":null,"position":6,"is_corresponding":false},{"id":563086,"name":"Janna H. Neltner","orcid":"0000-0002-2843-0411","position":7,"is_corresponding":false},{"id":685214,"name":"Jill Kolesar","orcid":"0000-0001-8575-4546","position":8,"is_corresponding":false},{"id":567101,"name":"John L. Villano","orcid":"0000-0001-5583-0093","position":9,"is_corresponding":false},{"id":948758,"name":"Ellen Reusch","orcid":null,"position":0,"is_corresponding":true}],"reference_count":46,"raw_metadata":null,"created_at":"2026-07-19T02:58:24.957414Z","pmid":"41199851","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":[]}