High-grade/large B-cell lymphoma with 11q aberration: A distinct molecularly defined entity in contemporary pathobiological classifications

<b>Purpose </b>High-grade/large B-cell lymphoma with 11q aberration (HGBCL-11q) is an aggressive mature B-cell lymphoma that mimics Burkitt lymphoma (BL) but lacks <i>MYC</i>-rearrangements. Correct diagnosis is essential to avoid misclassification and to support (future) risk-adapted management. <br/><b><br/>Methods </b>We reviewed recent classification updates, pivotal clinicopathologic reports, and genomic studies addressing HGBCL-11q. Accordingly, we summarized diagnostic criteria including histomorphology, immunophenotype and genomics, and collate reported clinical characteristics and treatment outcomes. <br/><b><br/>Results </b>HGBCL-11q is characterized by a chromosome 11q copy-number profile with a proximal gain/amplification at 11q23.2–q23.3 and a distal loss at 11q24.1–qter. Tumours typically show a germinal centre B-cell immunophenotype and BL-like histomorphology, yet genome-wide profiling supports assignment within the large B-cell lymphoma (LBCL) spectrum rather than true BL. HGBCL-11q predominantly affects children and adolescents and often presents with localized nodal disease in the head-and-neck region. In the largest multinational cohort of 72 pediatric patients, 3-year event-free and overall survival were 94 % and 96 %, respectively, with inferior outcomes mainly confined to patients with inborn and acquired errors of immunity or cancer predisposition syndromes. <br/><b><br/>Conclusion </b>HGBCL-11q is a genetically defined non-Hodgkin lymphoma with an excellent lymphoma-related prognosis under pediatric mature B-cell lymphoma–type regimens. Standardized testing for 11q aberration in cases of diffuse LBCL, HGBCL or BL morphology lacking <i>MYC</i>-rearrangements improves classification consistency and may enable chemoimmunotherapy de-escalation trials. Systematic evaluation for immunodeficiency and cancer predisposition syndromes is recommended to identify patients at risk and guide adapted therapy, supportive care and follow-up.