Lymphoma & related disorders

Mature B cell neoplasms

DLBCL and large B cell lymphomas with high grade features

Burkitt-like lymphoma with 11q aberration



Last staff update: 10 August 2022

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PubMed Search: Burkitt-like lymphoma with 11q aberration

Anna Shestakova, M.D., Ph.D.
Anamarija M. Perry, M.D.
Page views in 2021: 688
Page views in 2022 to date: 734
Cite this page: Shestakova A, Perry AM. Burkitt-like lymphoma with 11q aberration. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomaburkittlike11q.html. Accessed August 10th, 2022.
Definition / general
  • Rare B cell lymphoma that resembles Burkitt lymphoma morphologically, phenotypically and by gene expression profiling
  • Negative for MYC rearrangement
Essential features
  • Rare, germinal center derived, mature B cell lymphoma, characterized by proximal gains and telomeric losses of chromosome 11q
  • Features overlapping with Burkitt lymphoma but lacks MYC rearrangement
  • Most patients are children and young adults presenting with lymphadenopathy
  • Diffuse infiltrate of medium sized cells, frequently with interspersed tingible body macrophages and apoptotic debris
  • Lymphoma cells positive for CD20, CD10 and BCL6 and negative for BCL2, with Ki67 > 95%
  • Favorable prognosis with intensive chemoimmunotherapy
Terminology
  • Burkitt-like lymphoma with 11q aberration
  • Burkitt-like lymphoma
ICD coding
  • ICD-O: 9687/3 - Burkitt lymphoma, NOS
Epidemiology
Sites
Diagnosis
  • Tissue biopsy of lymph node or extranodal site
Prognostic factors
Case reports
Treatment
Microscopic (histologic) description
  • Diffuse involvement by sheets of lymphoma cells
  • Occasional cases show a vaguely nodular pattern (Haematologica 2019;104:1822)
  • Some cases are similar to Burkitt lymphoma, with tingible body macrophages, exhibiting starry sky pattern
  • Other cases have fewer macrophages but frequently show apoptotic bodies
  • Lymphoma cells are medium sized, have more cytological pleomorphism than typical Burkitt lymphoma, with more irregular nuclei
  • Some cases morphologically resemble diffuse large B cell lymphoma (Haematologica 2019;104:1822, Am J Clin Pathol 2017;149:17)
Microscopic (histologic) images

Contributed by Anna Shestakova, M.D., Ph.D.
Diffuse lymph node infiltration by lymphoma cells

Diffuse lymph node infiltration by lymphoma cells

Lymphoma cells with interspersed tingible body macrophages

Interspersed tingible body macrophages

Atypical lymphoma cells

Atypical lymphoma cells


CD20 stain

CD20 stain

CD10 stain

CD10 stain

BCL6 stain

BCL6 stain

Ki67 stain

Ki67 stain

Virtual slides

Images hosted on other servers:

Abdominal mass in a 20 year old man

Positive stains
Flow cytometry description
  • Mature B cell lymphoma with germinal center immunophenotype, positive for CD19, CD20, CD10 and CD38 with surface light chain restriction (Mod Pathol 2018;31:732)
Molecular / cytogenetics description
Sample pathology report
  • Lymph node, cervical, core needle biopsy:
    • Burkitt-like lymphoma with 11q aberration
Differential diagnosis
Board review style question #1

A 20 year old man presents with abdominal pain and recent 20 pound weight loss. He was found to have a 10 cm peripancreatic mass, which was biopsied and shown in the figure above. Cytogenetic studies are negative for MYC gene rearrangement. Chromosomal genetic microarray shows 11q aberration with proximal gain and telomeric loss. Which of the following is the typical immunophenotype seen in this entity?

  1. CD20+, CD10+, BCL6+, MUM1-, BCL2-, Ki67 > 95%
  2. CD20+, CD10+, BCL6+, MUM1+, BCL2+, Ki67 > 95%
  3. CD20+, CD10-, BCL6-, MUM1+, BCL2+, Ki67 > 95%
  4. CD20+, CD10-, BCL6-, MUM1+, BCL2-, Ki67 > 95%
Board review style answer #1
A. Burkitt-like lymphoma with 11q aberration usually shows the following immunophenotype: CD20+, CD10+, BCL6+, MUM1-, BCL2-, Ki67 > 95%

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Reference: Burkitt-like lymphoma with 11q aberration
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