Lymphoma & related disorders

Mature B cell neoplasms

Small B cell lymphomas with lymphoplasmacytic differentiation / marginal zone lymphomas

Pediatric nodal marginal zone lymphoma



Last author update: 25 July 2022
Last staff update: 25 July 2022

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PubMed Search: Pediatric nodal marginal zone lymphoma

Kathryn Gibbons, M.D.
Anamarija M. Perry, M.D.
Page views in 2021: 192
Page views in 2022 to date: 410
Cite this page: Gibbons K, Perry AM. Pediatric nodal marginal zone lymphoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/lymphomapediatricnodalMZL.html. Accessed August 17th, 2022.
Definition / general
  • Rare pediatric non-Hodgkin lymphoma, with characteristic morphologic and clinical features and a typically excellent prognosis
Essential features
  • Rare pediatric nodal lymphoma, more common in males
  • Typically localized to head and neck lymph nodes with excellent prognosis
  • Histologically, expanded marginal zones and interfollicular areas with expanded follicles
  • Polymorphous proliferation of monocytoid, centrocyte-like and plasmacytoid cells
ICD coding
  • ICD-O: 9699/3 - marginal zone B cell lymphoma, NOS
Epidemiology
Sites
  • Majority of patients have localized (stage I) head and neck lymphadenopathy
  • Inguinal / femoral lymph nodes involved in up to 20% of cases (Adv Anat Pathol 2017;24:128)
Pathophysiology
Etiology
  • Unknown
Clinical features
Diagnosis
Laboratory
Prognostic factors
Case reports
Treatment
Microscopic (histologic) description
  • Partial to complete effacement with sinusoidal obliteration (Adv Anat Pathol 2017;24:128)
  • Markedly expanded marginal zones with interfollicular infiltration of lymphoma cells
  • Diffuse areas often present (Virchows Arch 2016;468:141)
  • Often large follicles with extension of mantle zone into germinal centers, resembling progressive transformation of germinal centers; follicular colonization is sometimes seen (Pediatr Blood Cancer 2020;67:e28416)
  • Lymphoid infiltrate is polymorphous including monocytoid cells (small to medium sized, round nuclei, moderate cytoplasm), centrocyte-like cells (irregular nuclei, scant cytoplasm) and plasma cells (Adv Anat Pathol 2017;24:128)
Microscopic (histologic) images

Contributed by Kathryn Gibbons, M.D.

Lymph node with pediatric nodal marginal zone lymphoma

Atypical infiltrate


CD20 stain

PAX5 stain

CD43 stain

IgD stain

Virtual slides

Images hosted on other servers:

Pediatric nodal marginal zone lymphoma

Positive stains
Negative stains
Flow cytometry description
Molecular / cytogenetics description
Sample pathology report
  • Cervical lymph node, excisional biopsy:
    • Pediatric nodal marginal zone lymphoma
    • Histologic description: Sections show lymph node with effaced architecture by large nodules / follicles with markedly expanded marginal zones. Focally, there are large follicles that appear transformed with extension of mantle zones into germinal centers. Lymphoid infiltrate consists predominantly of small monocytoid appearing cells and scattered plasma cells.
    • Immunohistochemical stains show the atypical lymphoid cells to be positive for CD20, PAX5, CD43 and BCL2. They are negative for CD10 and BCL6. IgD highlights mantle zone expansion. Ki67 proliferative index is low (< 10%) in the atypical infiltrate.
    • Molecular studies are positive for clonal immunoglobulin heavy chain (IGH) gene rearrangement.
Differential diagnosis
Board review style question #1


An 18 year old man presents with localized nontender cervical lymphadenopathy. Excisional biopsy was performed and representative images are shown above. Flow cytometry showed a lambda light chain restricted B cell population, while molecular analysis shows a clonal IGH rearrangement. What is the most likely diagnosis?

  1. Adult marginal zone lymphoma
  2. Atypical marginal zone hyperplasia
  3. Pediatric nodal marginal zone lymphoma
  4. Pediatric type follicular lymphoma
  5. Progressive transformation of germinal centers
Board review style answer #1
C. Pediatric nodal marginal zone lymphoma. The images show a lymph node with architecture effaced by atypical lymphoid proliferation composed of large follicles and extension of mantle zones into the germinal centers, mimicking progressive transformation of germinal centers. On higher magnification, the lymphoid infiltrate expands the marginal zone and extends into the interfollicular area. Flow cytometry and molecular studies support a lymphoma diagnosis over a benign process. Given the clinical history of a young male with localized nontender cervical lymphadenopathy, morphologic findings, in conjunction with ancillary studies, are most consistent with pediatric nodal marginal zone lymphoma.

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Reference: Pediatric nodal marginal zone lymphoma
Board review style question #2
Which panel of immunohistochemical stains is most consistent with pediatric marginal zone lymphoma?

  1. CD20+, CD10+, BCL6+, BCL2+
  2. CD20+, CD43+, CD10-, BCL6-, BCL2+
  3. CD20+, CD43-, CD10-, BCL2-, CD5-
  4. CD20-, CD3+, PD-1+
  5. CD20+, CD10+, BCL6+, BCL2-
Board review style answer #2
B. CD20+, CD43+, CD10-, BCL6-, BCL2+. In pediatric nodal marginal zone lymphoma, the lymphoma cells are neoplastic B cells, positive for CD20, CD43 and BCL2. They are not derived from a germinal center and are negative for CD10 and BCL6. Therefore, answer B is the best choice. Answer A is consistent with lymphoma of germinal center origin. The stains in answer C are nonspecific. Stains in answer D are supportive of a T cell process. The stains in answer E can be seen in reactive follicular hyperplasia, among other diagnoses.

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Reference: Pediatric nodal marginal zone lymphoma
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