Childhood nodal marginal zone lymphoma with unusual clinicopathologic and cytogenetic features for the pediatric variant: a case report

Pediatr Dev Pathol. Mar-Apr 2015;18(2):167-71. doi: 10.2350/14-12-1584-CR.1. Epub 2015 Jan 27.


Nodal marginal zone lymphoma (NMZL) is a B-cell lymphoma that shares morphologic and immunophenotypic features with extranodal and splenic marginal zone lymphomas but lacks extranodal or splenic involvement at presentation. NMZL occurs mostly in adults with no sex predilection, at advanced stage (III or IV), with frequent relapses and a high incidence of tumoral genetic abnormalities including trisomies 3 and 18 and gain of 7q. Pediatric NMZL, however, is a rare but distinct variant of NMZL with characteristic features including male predominance, asymptomatic and localized (stage I) disease, low relapse rates with excellent outcomes, and a lower incidence of essentially similar genetic aberrations compared to adult NMZL. Here we describe a unique case of childhood NMZL with unusual clinicopathologic features for the pediatric variant including generalized lymphadenopathy, high-stage disease with persistence after therapy, unusual immunophenotype (CD5, CD23, and BCL6 positive), and unique chromosomal abnormalities including monosomy 20 and add(10)(p11.2).

Keywords: NMZL; atypical; child; lymphoma; nodal marginal zone lymphoma; pediatric.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Biomarkers, Tumor / genetics*
  • Biopsy
  • Chromosome Aberrations*
  • Chromosomes, Human, Pair 10 / genetics*
  • Chromosomes, Human, Pair 20 / genetics*
  • Female
  • Genetic Predisposition to Disease
  • Humans
  • Immunohistochemistry
  • Immunophenotyping
  • In Situ Hybridization, Fluorescence
  • Lymphoma, B-Cell, Marginal Zone / genetics*
  • Lymphoma, B-Cell, Marginal Zone / immunology
  • Lymphoma, B-Cell, Marginal Zone / pathology*
  • Lymphoma, B-Cell, Marginal Zone / therapy
  • Monosomy
  • Neoplasm Staging
  • Phenotype
  • Predictive Value of Tests
  • Recurrence
  • Time Factors
  • Treatment Outcome


  • Biomarkers, Tumor