ArticleJournal of clinical oncology : official journal of the American Society of Clinical Oncology2008
Del(6)(q22) and BCL6 rearrangements in primary CNS lymphoma are indicators of an aggressive clinical course.
Article in Journal of clinical oncology : official journal of the American Society of Clinical Oncology, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 40 papers.
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Who cites it
40 citing papers in PubMed, 117 citations in OpenAlex.
- MYC protein expression in primary diffuse large B-cell lymphoma of the central nervous system.PloS one · 2014Trial
- Genetic orchestration of the primary central nervous system lymphoma microenvironment.Biomarker research · 2026Review
- Clinicopathological, Genomic, and Transcriptomic Feature Analysis of Primary Adrenal Large B-cell Lymphoma: Insights Into Immune-privileged Sites.The American journal of surgical pathology · 2025Article
- Protein tyrosine phosphatase receptor type kappa (PTPRK) revisited: evolving insights into structure, function, and pathology.Journal of translational medicine · 2025Review
- Detection of diagnostic somatic copy number alterations from cerebrospinal fluid cell-free DNA in brain tumor patients.Acta neuropathologica communications · 2024Article
- Double-hit primary central nervous system lymphoma with histogenetically proven bone marrow infiltration: a case report and a review of the literature.Brain tumor pathology · 2024Review
- The Molecular Landscape of Primary CNS Lymphomas (PCNSLs) in Children and Young Adults.Cancers · 2024Article
- Primary central nervous system lymphoma.Nature reviews. Disease primers · 2023Review
- Double-hit and double-expressor primary central nervous system lymphoma: Experience from North India of an infrequent but aggressive variant.Surgical neurology international · 2023Article
- Two cases of primary diffuse large B-cell lymphoma of the CNS associated with t(8;14)(q24;q32) or t(3;14)(q27;q32) identified by G-banding and fluorescence in situ hybridization applied to metaphase spreads.Journal of clinical and experimental hematopathology : JCEH · 2022Article
- B-cell Lymphoma 6 (BCL6): From Master Regulator of Humoral Immunity to Oncogenic Driver in Pediatric Cancers.Molecular cancer research : MCR · 2022Review
- Review
- Clinical Outcomes of RTOG 9310 Protocol for Primary Central Nervous System Lymphoma: Single-Center Experience with 87 Patients.Current oncology (Toronto, Ont.) · 2021Article
- Case Report: Identification of Potential Prognosis-RelatedFrontiers in endocrinology · 2021Article
- Complete Blood Count Score Model Predicts Inferior Prognosis in Primary Central Nervous System Lymphoma.Frontiers in oncology · 2021Article
- Primary Central Nervous System Lymphomas: A Diagnostic Overview of Key Histomorphologic, Immunophenotypic, and Genetic Features.Diagnostics (Basel, Switzerland) · 2020Review
- Molecular features of a large cohort of primary central nervous system lymphoma using tissue microarray.Blood advances · 2019Article
- Rare cases of primary central nervous system anaplastic variant of diffuse large B-cell lymphoma.Diagnostic pathology · 2019Article
- Primary Central Nervous System Lymphoma: Molecular Pathogenesis and Advances in Treatment.Translational oncology · 2019Review
- The impact ofInternational journal of clinical and experimental pathology · 2019Article
Corrections and comments
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Authors and funding
11 authors at 2 institutions in 1 country.
Funding
Abstract
purposePrimary CNS lymphoma (PCNSL) is an aggressive lymphoma but clinically validated biologic markers that can predict natural history to tailor treatment according to risk are lacking. Several genetic changes including BCL6 rearrangements and deletion of 6q22, containing the putative tumor suppressor gene PTPRK, are potential risk predictors. Herein we determined the prevalence and survival impact of del(6)(q22) and BCL6, immunoglobulin heavy chain (IGH), and MYC gene rearrangements in a large PCNSL cohort treated in a single center. PATIENTS AND
methodsInterphase fluorescence in situ hybridization was performed using two-color probes for BCL6, MYC, IGH-BCL6, and del(6)(q22) on thin sections of 75 paraffin-embedded samples from 75 HIV-negative, immunocompetent patients newly diagnosed with PCNSL. Survival data were analyzed using Kaplan-Meier survival curves, log-rank tests, and proportional hazards regression adjusting for age, deep structure involvement, and high-dose methotrexate (HDMTX) treatment.
resultsThe prevalence of del(6)(q22) and BCL6, IGH, and MYC translocations was 45%,17%, 13%, and 3%, respectively. The presence of del(6)(q22) and/or a BCL6 translocation was associated with inferior overall survival (OS; P = .0097). The presence of either del(6)(q22) alone or a BCL6 translocation alone was also associated with inferior OS (P = .0087). Univariable results held after adjusting for age, deep structure involvement, and HDMTX.
conclusionDel (6)(q22) and BCL6 rearrangements are common in PCNSL and predict for decreased OS independent of deep structure involvement and HDMTX. Unlike systemic diffuse large B-cell lymphoma, del(6)(q22) is common and IGH translocations are infrequent and usually involve BCL6 rather than BCL2, suggesting a distinct pathogenesis.
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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.