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Genomics of Burkitt-like lymphoma/high-grade B-cell lymphoma with 11q aberrations
Source: Childhood Cancer Genomics (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 30, 2025 · Captured 2026-09-09.
Selected source text with whitespace normalised. This Triangle page is not an NCI PDQ summary. Independent clinical review is pending.
Context: Non-Hodgkin Lymphoma / Mature B-cell Lymphoma / Burkitt lymphoma
Burkitt-like lymphoma with 11q aberration was added as a provisional entity in the 2017 revised WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues.[13] In the 5th edition of the WHO classification, this entity was renamed high-grade B-cell lymphoma with 11q aberrations.[14] In this entity, MYC rearrangement is absent, and the characteristic chromosome 11q finding (detected cytogenetically and/or with copy-number DNA arrays) is 11q23.2-q23.3 gain/amplification and 11q24.1-qter loss.[15,16]
In a study of 102 lymphomas that morphologically resembled Burkitt lymphoma, diffuse large B-cell lymphoma, and high-grade B-cell lymphoma, unclassifiable, 13 cases (13%) lacked a MYC rearrangement but were positive for 11q proximal gain and telomeric loss by fluorescence in situ hybridization.[17]
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Most patients with high-grade B-cell lymphoma with 11q aberrations present in the adolescent and young adult age range with localized nodal disease.[16,17] Head and neck involvement is the most common presentation, although presentation in other nodal areas, as well as in the abdomen, can occur.
Cases show a very high proliferative index and can show a focal starry sky pattern.[16,17]
Outcomes appear highly favorable in the small number of cases identified.[16,17]
The variant landscape of high-grade B-cell lymphoma with 11q aberrations is distinct from that of Burkitt lymphoma. Variants commonly observed in Burkitt lymphoma (e.g., ID3, TCF3, and CCND3) are uncommon in high-grade B-cell lymphoma with 11q aberrations.[15] Conversely, variants in GNA13 appear to be common (up to 50%) in patients with high-grade B-cell lymphoma with 11q aberrations and are less common in patients with Burkitt lymphoma.
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For information about the treatment of childhood Burkitt lymphoma, see Childhood Non-Hodgkin Lymphoma Treatment.
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Preserved source evidence · Independent clinical review pending · Not medical advice