2009
DOI: 10.3324/haematol.2009.013177
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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: BackgroundPediatric 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. Design and MethodsWe analyzed 25 cases of pediatric follicular lymphoma (patients aged ≤18 years) by morphology, immunohistochemistry and interphase fluorescence in situ hybridization. All patients analyzed were treated within Non-… Show more

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Cited by 104 publications
(133 citation statements)
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“…This is likely due to the fact that IRF4 translocations occur mainly in pediatric or young adult patients (15% versus 2% IRF4 translocations in adult FL patients 18 ), while the median age of the patients analyzed for IRF4 translocations in our sample was 60 years (range, 41 to 80 years; data not shown) and, indeed, only one pediatric patient (aged 9 years) was included in this study. Consistent with recent findings, this child with FL3U (ID31, Online Supplementary Table S1) did not have a translocation of BCL2, showed no BCL2 protein expression, 4 and had a CD10 -IRF4/MUM1 -immunophenotype: IRF4-translocation analysis was not, however, successful in this particular case.…”
Section: Discussionsupporting
confidence: 73%
See 1 more Smart Citation
“…This is likely due to the fact that IRF4 translocations occur mainly in pediatric or young adult patients (15% versus 2% IRF4 translocations in adult FL patients 18 ), while the median age of the patients analyzed for IRF4 translocations in our sample was 60 years (range, 41 to 80 years; data not shown) and, indeed, only one pediatric patient (aged 9 years) was included in this study. Consistent with recent findings, this child with FL3U (ID31, Online Supplementary Table S1) did not have a translocation of BCL2, showed no BCL2 protein expression, 4 and had a CD10 -IRF4/MUM1 -immunophenotype: IRF4-translocation analysis was not, however, successful in this particular case.…”
Section: Discussionsupporting
confidence: 73%
“…1 Roughly 85% of these tumors are associated with the t(14;18)(q21;q32) chromosome translocation that constitutively deregulates and over-expresses the BCL2 proto-oncogene. However, rarer subtypes of FL, often arising primarily in extranodal sites, in the pediatric population, or with varying architectural or cytological features, frequently display distinct clinical and/or cytogenetic features [1][2][3][4] in comparison with the classic nodal FL grade 1 (FL1) and 2 (FL2). Of these subtypes, FL grade 3 (FL3) have gained considerable interest because of their unresolved status as indolent or aggressive neoplasms, and their varying immunophenotypic, cytogenetic and possibly clinical features.…”
Section: Discussionmentioning
confidence: 99%
“…The t(14;18) translocation or BCL6 rearrangements are uncommon, although BCL2 protein expression may be found in a subset of the tumors. 83 Recurrent breaks in the IGH@ gene are seen in several cases, but the corresponding partners have not been identified. [83][84][85] Some patients have had long survival with only local treatment, and the most appropriate management of these patients is yet to be defined.…”
Section: Clinical Features In Disease Definitionmentioning
confidence: 99%
“…Interestingly, a coexisting DLBCL component does not appear to alter the prognosis in pediatric FL, unlike in adult cases. 27 Pediatric FL appears to show a favorable clinical course. Interestingly, a recent study has identified frequent IRF4/MUM1 translocations in cases of FL and DLBCL occurring in childhood and young adults.…”
Section: Variant-pediatric Flmentioning
confidence: 92%
“…3 Specifically, pediatric FL shows an increased proportion of cases with focal disease, grade 3 morphology, lack of BCL2 protein by immunohistochemistry, and lack of the IGH/BCL2 translocation. 27,28 In light of these findings, it is often challenging to distinguish between florid follicular hyperplasia and pediatric FL. It should be remembered that follicular hyperplasia may sometimes yield a clonal B-cell population by PCR or even by flow cytometry.…”
Section: Variant-pediatric Flmentioning
confidence: 99%