PMID- 20305621
OWN - NLM
STAT- MEDLINE
DCOM- 20100903
LR  - 20230210
IS  - 1530-0285 (Electronic)
IS  - 0893-3952 (Print)
IS  - 0893-3952 (Linking)
VI  - 23
IP  - 6
DP  - 2010 Jun
TI  - Marginal zone lymphomas in children and the young adult population; 
      characterization of genetic aberrations by FISH and RT-PCR.
PG  - 866-73
LID - 10.1038/modpathol.2010.63 [doi]
AB  - Marginal zone lymphomas present rarely in children and young adults as either 
      primary nodal or extranodal disease and have an excellent prognosis. To date, 
      chromosomal aberrations have not been analyzed in the pediatric and young adult 
      population. We undertook a study to analyze genetic alterations in nodal and 
      extranodal marginal zone lymphomas in children and young adults using 
      fluorescence in situ hybridization (FISH) and RT-PCR. These findings were 
      correlated with clinical features at presentation and immunophenotype. Forty-one 
      cases were identified meeting these criteria. The age range was 1.5-29 years old 
      with 49% of the cases <18 years of age. 73% of the marginal zone lymphoma cases 
      showed evidence of light chain restriction by immunohistochemistry or flow 
      cytometry. CD43 was coexpressed in 83%. 85% of the marginal zone lymphoma cases 
      tested showed evidence of immunoglobulin heavy chain gene rearrangement. 
      Fifty-nine percent of the cases were nodal marginal zone lymphomas with a median 
      age at presentation of 16 years and an M/F ratio of 7:1. Twenty-one percent of 
      the nodal marginal zone lymphoma cases contained genetic aberrations. Seventeen 
      percent contained trisomy 18 with one case containing an additional trisomy 3. A 
      translocation of the immunoglobulin heavy chain gene to an unknown partner gene 
      was present in one case. Forty-one percent of the cases were extranodal marginal 
      zone lymphomas with a median age of 24 years and a M/F ratio of 1.4:1. Eighteen 
      percent of the extranodal marginal zone lymphoma cases contained genetic 
      aberrations. The t(14;18) involving the IGH and MALT1 genes was present in one 
      case, tetraploidy was present in one case, and another case contained trisomy 3. 
      Overall the incidence of genetic aberrations in marginal zone lymphomas in the 
      pediatric and young adult population is low, but the aberrations seen are similar 
      to those seen in the adult population.
FAU - Rizzo, Kathryn A
AU  - Rizzo KA
AD  - Hematopathology Section, Laboratory of Pathology, Center for Cancer Research, 
      National Cancer Institute, National Institutes of Health, Bethesda, MD 
      20892-1500, USA.
FAU - Streubel, Berthold
AU  - Streubel B
FAU - Pittaluga, Stefania
AU  - Pittaluga S
FAU - Chott, Andreas
AU  - Chott A
FAU - Xi, Liqiang
AU  - Xi L
FAU - Raffeld, Mark
AU  - Raffeld M
FAU - Jaffe, Elaine S
AU  - Jaffe ES
LA  - eng
GR  - Z01 SC000550-27/ImNIH/Intramural NIH HHS/United States
PT  - Journal Article
DEP - 20100319
PL  - United States
TA  - Mod Pathol
JT  - Modern pathology : an official journal of the United States and Canadian Academy 
      of Pathology, Inc
JID - 8806605
RN  - 0 (Immunoglobulin Light Chains)
RN  - 0 (Leukosialin)
RN  - 0 (Neoplasm Proteins)
RN  - 0 (SPN protein, human)
RN  - EC 3.4.22.- (Caspases)
RN  - EC 3.4.22.- (MALT1 protein, human)
RN  - EC 3.4.22.- (Mucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 Protein)
SB  - IM
MH  - Adolescent
MH  - Caspases/genetics
MH  - Child
MH  - Child, Preschool
MH  - *Chromosome Aberrations
MH  - Female
MH  - Flow Cytometry
MH  - *Gene Expression Regulation, Neoplastic
MH  - Gene Rearrangement
MH  - Genes, Immunoglobulin Heavy Chain
MH  - Humans
MH  - Immunoglobulin Light Chains/analysis
MH  - Immunohistochemistry
MH  - Immunophenotyping
MH  - *In Situ Hybridization, Fluorescence
MH  - Infant
MH  - Leukosialin/analysis
MH  - Lymphoma, B-Cell, Marginal Zone/*genetics/immunology/pathology
MH  - Male
MH  - Mucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 Protein
MH  - Neoplasm Proteins/genetics
MH  - *Reverse Transcriptase Polymerase Chain Reaction
MH  - Translocation, Genetic
MH  - Young Adult
PMC - PMC6329460
MID - NIHMS1004218
COIS- Disclosure/conflict of interest The authors declare no conflict of interest.
EDAT- 2010/03/23 06:00
MHDA- 2010/09/04 06:00
PMCR- 2019/01/11
CRDT- 2010/03/23 06:00
PHST- 2010/03/23 06:00 [entrez]
PHST- 2010/03/23 06:00 [pubmed]
PHST- 2010/09/04 06:00 [medline]
PHST- 2019/01/11 00:00 [pmc-release]
AID - S0893-3952(22)02705-3 [pii]
AID - 10.1038/modpathol.2010.63 [doi]
PST - ppublish
SO  - Mod Pathol. 2010 Jun;23(6):866-73. doi: 10.1038/modpathol.2010.63. Epub 2010 Mar 
      19.