title: Pediatric follicular lymphoma--a clinico-pathological study of a population-based series of patients treated within the Non-Hodgkin's Lymphoma--Berlin-Frankfurt-Munster (NHL-BFM) multicenter trials.
abstract: Background Pediatric follicular lymphoma has recently been recognized as a novel variant of follicular lymphoma in the World Health Organization classification of lymphomas. Given the rarity of the disease, histopathological and genetic data on this type of lymphoma are still scarce.
  
  
  METHODS
  We analyzed 25 cases of pediatric follicular lymphoma (patients aged <or=18 years) by morphology, immunohistochemistry and interphase fluorescence in situ hybridization. All patients analyzed were treated within Non-Hodgkin's Lymphoma--Berlin-Frankfurt-Münster (NHL-BFM) multicenter trials, and the cohort was representative of the German population.
  
  
  RESULTS
  The genetic hallmark of adult follicular lymphoma, t(14;18)(q32;q21), was not detectable in any of the pediatric cases, although BCL2 protein was expressed in 55% of the latter cases. No correlation was found between BCL2 protein expression and outcome. Chromosomal breaks in the immunoglobulin heavy chain gene (IGH) and the BCL6 locus were detected in 5 of 17 and 1 of 18 cases, respectively. Patients with pediatric follicular lymphoma had long event-free survival and, in contrast to adult follicular lymphoma, the clinical course was not dominated by relapses. A simultaneous diffuse large B-cell lymphoma was frequently detected at initial diagnosis in children but did not indicate an aggressive clinical course. Conclusions Our data suggest that pediatric follicular lymphoma is a disease that differs from its adult counterpart both genetically and clinically.
authors.affiliation: 1Department of Pathology, Hematopathology Section and Lymph Node Registry, Christian-Albrechts University, Kiel, Germany.
authors.forename: Ilske
authors.initials: I
authors.lastname: Oschlies
authors.forename: Itziar
authors.initials: I
authors.lastname: Salaverria
authors.forename: Friederike
authors.initials: F
authors.lastname: Mahn
authors.forename: Andrea
authors.initials: A
authors.lastname: Meinhardt
authors.forename: Martin
authors.initials: M
authors.lastname: Zimmermann
authors.forename: Wilhelm
authors.initials: W
authors.lastname: Woessmann
authors.forename: Birgit
authors.initials: B
authors.lastname: Burkhardt
authors.forename: Stefan
authors.initials: S
authors.lastname: Gesk
authors.forename: Matthias
authors.initials: M
authors.lastname: Krams
authors.forename: Alfred
authors.initials: A
authors.lastname: Reiter
authors.forename: Reiner
authors.initials: R
authors.lastname: Siebert
authors.forename: Wolfram
authors.initials: W
authors.lastname: Klapper
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D020732:Cytogenetic Analysis; D005260:Female; D015870:Gene Expression; D006801:Humans; D007150:Immunohistochemistry; D007223:Infant; D008224:Lymphoma, Follicular / Q000235:genetics* / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D016403:Lymphoma, Large B-Cell, Diffuse / Q000175:diagnosis; D008297:Male; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000235:genetics; D016896:Treatment Outcome
chemical_list: D019253:Proto-Oncogene Proteins c-bcl-2
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