Lymphoma & related disorders
Mature B cell neoplasms
Small B cell lymphomas with lymphoplasmacytic differentiation / marginal zone lymphomas
Marginal zone-nodal

Topic Completed: 1 March 2011

Minor changes: 18 December 2020

Copyright: 2001-2021,, Inc.

PubMed Search: nodal marginal zone lymphoma

Nikhil Sangle, M.D.
Page views in 2020: 11,254
Page views in 2021 to date: 2,385
Cite this page: Sangle N. Marginal zone-nodal. website. Accessed March 4th, 2021.
Definition / general
  • Uncommon low grade lesion, associated with Sjogren syndrome, that resembles MALT lymphoma with monocytoid, centrocyte-like cells
  • For diagnosis, must rule out evidence of extranodal or splenic disease
Clinical features
  • Uncommon
  • Similar incidence in men and women
  • Mean age 61 years (range 26 - 92 years)
  • Usually associated with Sjogren syndrome
  • Associated with Hepatitis C in some studies (Br J Haematol 2007;136:301)
  • Usually localized lymphadenopathy (59% stage I - II at diagnosis)
  • Marrow and blood involvement are rare
  • Usually indolent; 5 year survival is 57 - 79%; may be longer than extranodal marginal cell lymphoma
  • May be preceded by marginal zone hyperplasia with clear cells (Am J Clin Pathol 2001;116:550)

MALT subtype:
  • Perisinusoidal, perivascular infiltration of monocytoid / centrocytic cells and residual germinal centers with well preserved mantle cuff
  • Negative for IgD, cyclin D1, CD5; 44%
  • Had masses in salivary gland, soft tissue, skin or breast suggestive of extranodal lymphoma (Am J Surg Pathol 1999;23:59)

Splenic subtype:
  • Polymorphic infiltrate surrounding residual germinal centers, no or attenuated mantle cuff
  • BCL2+ (83%); IgD+ (100%), CD5-, CD23-, cyclinD1-
  • Resembles follicular lymphoma, but residual germinal centers were BCL2-, no t(14;18); no splenomegaly (Am J Surg Pathol 1999;23:59)
Features in Children / Young Adults
  • 67% of marginal zone B cell lymphomas in children / young adults (up to age 29 years) are nodal, usually involving head and neck nodes
  • 80% male, usually no underlying disease
  • Excellent prognosis with low risk of recurrence
  • Have partial to total effacement of lymph nodes with obliteration of sinuses by atypical cells with predominantly interfollicular distribution and marked expansion of marginal zones; disruption of residual follicles in 2/3 resembles progressive transformation of germinal centers; cells are polymorphic, small to medium size, some with moderate cytoplasm and round nuclei and others with scant cytoplasm and irregular nuclei; frequent plasma cells and larger transformed cells, few / rare blast cells (Am J Surg Pathol 2003;27:522)
Prognostic factors
Case reports
Microscopic (histologic) description
  • Partial / total effacement of architecture, interfollicular infiltrate of monocytoid, centrocyte-like B cells that are 2 - 3× larger than small lymphocytes
  • Tumor cells surround and infiltrate follicles, have moderately abundant pale cytoplasm, round / irregular nuclei with clumped chromatin
  • Similar morphology to MALT lymphoma or splenic marginal zone B cell lymphoma
  • Also has benign follicular centers
  • 30% have neoplastic plasma cells
  • Presence of follicular dendritic meshwork remnant suggestive of colonized follicles favors diagnosis of marginal zone lymphoma
Positive stains
Negative stains
Molecular / cytogenetics description
  • Clonal rearrangements of IgH and light chains
  • Trisomy 18 and 3; 1q21 or 1q34 abnormalities
Differential diagnosis
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