{"doi":"10.1093/neuonc/noad033","title":"Clinical, histological, and molecular features of gliomas in adults with neurofibromatosis type 1","abstract":"BACKGROUND: People with NF1 have an increased prevalence of central nervous system malignancy. However, little is known about the clinical course or pathologic features of NF1-associated gliomas in adults, limiting clinical care and research. METHODS: Adults (≥18 years) with NF1 and histologically confirmed non-optic pathway gliomas (non-OPGs) at Johns Hopkins Hospital, Memorial Sloan Kettering Cancer Center, and Washington University presenting between 1990 and 2020 were identified. Retrospective data were collated, and pathology was reviewed centrally. RESULTS: Forty-five patients, comprising 23 females (51%), met eligibility criteria, with a median of age 37 (18-68 years) and performance status of 80% (30%-100%). Tissue was available for 35 patients. Diagnoses included infiltrating (low-grade) astrocytoma (9), glioblastoma (7), high-grade astrocytoma with piloid features (4), pilocytic astrocytoma (4), high-grade astrocytoma (3), WHO diagnosis not reached (4) and one each of gliosarcoma, ganglioglioma, embryonal tumor, and diffuse midline glioma. Seventy-one percent of tumors were midline and underwent biopsy only. All 27 tumors evaluated were IDH1-wild-type, independent of histology. In the 10 cases with molecular testing, the most common genetic variants were NF1, EGFR, ATRX, CDKN2A/B, TP53, TERT, and MSH2/3 mutation. While the treatments provided varied, the median overall survival was 24 months [2-267 months] across all ages, and 38.5 [18-109] months in individuals with grade 1-2 gliomas. CONCLUSIONS: Non-OPGs in adults with NF1, including low-grade tumors, often have an aggressive clinical course, indicating a need to better understand the pathobiology of these NF1-associated gliomas.","journal":"Neuro-Oncology","year":2023,"id":323889,"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":35,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.8396,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2023-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":281415,"name":"Anna F. Piotrowski","orcid":"0000-0003-0058-8476","position":1,"is_corresponding":false},{"id":225598,"name":"Jian Campian","orcid":"0000-0002-5317-8481","position":2,"is_corresponding":false},{"id":1039259,"name":"Jose Diarte","orcid":"0000-0001-6672-8617","position":3,"is_corresponding":false},{"id":288108,"name":"Fausto J. Rodríguez","orcid":"0000-0001-8662-1219","position":4,"is_corresponding":false},{"id":236579,"name":"Tejus Bale","orcid":"0000-0003-3641-9287","position":5,"is_corresponding":false},{"id":259979,"name":"Sonika Dahiya","orcid":"0000-0002-5585-0964","position":6,"is_corresponding":false},{"id":295596,"name":"David H. Gutmann","orcid":"0000-0002-3127-5045","position":7,"is_corresponding":false},{"id":363766,"name":"Calixto‐Hope G. Lucas","orcid":"0000-0002-8347-9592","position":8,"is_corresponding":false},{"id":757157,"name":"Laura Prichett","orcid":"0000-0002-6029-6902","position":9,"is_corresponding":false},{"id":87670,"name":"Ingo K. Mellinghoff","orcid":"0000-0002-4347-8149","position":10,"is_corresponding":false},{"id":80025,"name":"Jaishri O. Blakeley","orcid":"0000-0002-1049-0993","position":11,"is_corresponding":false},{"id":422296,"name":"Carlos G. Romo","orcid":"0000-0002-0260-7047","position":0,"is_corresponding":true}],"reference_count":41,"raw_metadata":null,"created_at":"2026-07-19T01:08:06.496579Z","pmid":"36840626","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":[]}