title: Diffuse large B-cell lymphoma in pediatric patients belongs predominantly to the germinal-center type B-cell lymphomas: a clinicopathologic analysis of cases included in the German BFM (Berlin-Frankfurt-Munster) Multicenter Trial.
abstract: Diffuse large B-cell lymphoma (DLBCL) in adults is a heterogeneous disease. Biologic subgroups of DLBCL with a favorable prognosis (germinal center B-cell-like, GCB) and with a poor prognosis (activated B-cell-like, ABC) have been defined by gene expression profiling and can be distinguished by immunohistochemistry. In contrast to their adult counterparts, children with DLBCL have an excellent prognosis. We analyzed 63 cases of DLBCL in pediatric patients by immunohistochemistry and fluorescence in situ hybridization (FISH) and found a striking predominance of a GCB subtype, which might explain the good clinical outcome in these lymphomas. Interestingly, FISH applied to 50 of these cases, as well as conventional cytogenetics available in 3 cases, revealed absence of the translocation t(14;18) involving the BCL2 gene, which is present in about 15% of adult GCB subtype DLBCL. Our data indicate that pediatric DLBCL differs from adult DLBCL and might comprise a biologically unique subgroup of DLBCL from which important insights into the pathogenesis and biology of this disease might be gained.
authors.affiliation: Institute of Pathology, Department of Hematopathology and Lymph Node Registry Kiel, Niemannsweg 11, 24105 Kiel, Germany.
authors.forename: Ilske
authors.initials: I
authors.lastname: Oschlies
authors.forename: Wolfram
authors.initials: W
authors.lastname: Klapper
authors.forename: Martin
authors.initials: M
authors.lastname: Zimmermann
authors.forename: Matthias
authors.initials: M
authors.lastname: Krams
authors.forename: Hans-Heinrich
authors.initials: HH
authors.lastname: Wacker
authors.forename: Birgit
authors.initials: B
authors.lastname: Burkhardt
authors.forename: Lana
authors.initials: L
authors.lastname: Harder
authors.forename: Reiner
authors.initials: R
authors.lastname: Siebert
authors.forename: Alfred
authors.initials: A
authors.lastname: Reiter
authors.forename: Reza
authors.initials: R
authors.lastname: Parwaresch
mesh_terms: D002648:Child; D002675:Child, Preschool; D002883:Chromosomes, Human, Pair 14; D002887:Chromosomes, Human, Pair 18; D005260:Female; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007223:Infant; D007945:Leukemia, Lymphoid / Q000145:classification* / Q000235:genetics / Q000401:mortality / Q000473:pathology; D016393:Lymphoma, B-Cell / Q000145:classification* / Q000235:genetics / Q000401:mortality / Q000473:pathology; D008297:Male; D016019:Survival Analysis; D014178:Translocation, Genetic