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.
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.
Oschlies
Ilske
I
1Department of Pathology, Hematopathology Section and Lymph Node Registry, Christian-Albrechts University, Kiel, Germany.
Salaverria
Itziar
I
Mahn
Friederike
F
Meinhardt
Andrea
A
Zimmermann
Martin
M
Woessmann
Wilhelm
W
Burkhardt
Birgit
B
Gesk
Stefan
S
Krams
Matthias
M
Reiter
Alfred
A
Siebert
Reiner
R
Klapper
Wolfram
W
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
D019253:Proto-Oncogene Proteins c-bcl-2
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