Genetic aberrations in pediatric follicular lymphoma
Details
Serval ID
serval:BIB_FA4631F9A13C
Type
Inproceedings: an article in a conference proceedings.
Publication sub-type
Abstract (Abstract): shot summary in a article that contain essentials elements presented during a scientific conference, lecture or from a poster.
Collection
Publications
Institution
Title
Genetic aberrations in pediatric follicular lymphoma
Title of the conference
4th International Symposium on Childhood, Adolescent and Young Adult Non-Hodgkin's Lymphoma
Address
New York, United-States, November 1-3, 2012
ISBN
0007-1048
Publication state
Published
Issued date
2012
Volume
159
Series
British Journal of Haematology
Pages
20
Language
english
Notes
Document Type: Meeting Abstract
Abstract
Background: Pediatric follicular lymphoma (FL) is a rare disease that
differs from its adult counterpart both genetically and clinically.
Excluding pediatric FL with IRF4-translocation, the genetic events
associated with pediatric FL have not yet been defined.
Objectives: The aim of this study was to perform a complete genetic
characterization of IRF4-translocation negative pediatric follicular
lymphomas to elucidate the genetic profile of these rare pediatric cases
and determine common genetic alterations that could be associated to
this phenotype.
Design/Methods: We applied array-comparative genomic hybridization
and molecular inversion probe assay adapted to formalin-fixed
paraffin-embedded tissues from 18 patients aged £18 years diagnosed
with FL. With the exception of one case with only focal involvement by
lymphoma, the tumor cell content exceeded 50% in the evaluable
samples. Eleven of 18 patients were treated according to NHL-BFM
group multicenter trials whereas the remaining according to different
protocols. All lacked t(14;18) translocation. Mutational analysis of
TNFRSF14 gene was performed in 17 cases.
Results: Only six pediatric cases displayed chromosomal imbalances,
with gain/amplification of 6pter-p24.3 (including IRF4) and deletion/
copy number neutral-loss of heterozygosity in 1p36 (including
TNFRSF14) being the most frequent alterations. Sequencing of the
candidate gene TNFRSF14 at 1p36.32 showed nine mutations in seven
cases.
Conclusion: Combination of molecular and genetic features differentiated
a recurrent pattern of genomic imbalances as well as of
TNFRSF14 mutations in pediatric FL which together with other genetic
alterations distinguishes two subsets of pediatric follicular lymphomas.
The first group shows genomic aberrations and is associated with more
aggressive histopathologic and clinical features. The second group lacks
genetic alterations detectable with the present approaches and is
associated with a more limited disease. Despite the absence of genomic
aberrations, these cases resembled FL by their histopathological
features.
differs from its adult counterpart both genetically and clinically.
Excluding pediatric FL with IRF4-translocation, the genetic events
associated with pediatric FL have not yet been defined.
Objectives: The aim of this study was to perform a complete genetic
characterization of IRF4-translocation negative pediatric follicular
lymphomas to elucidate the genetic profile of these rare pediatric cases
and determine common genetic alterations that could be associated to
this phenotype.
Design/Methods: We applied array-comparative genomic hybridization
and molecular inversion probe assay adapted to formalin-fixed
paraffin-embedded tissues from 18 patients aged £18 years diagnosed
with FL. With the exception of one case with only focal involvement by
lymphoma, the tumor cell content exceeded 50% in the evaluable
samples. Eleven of 18 patients were treated according to NHL-BFM
group multicenter trials whereas the remaining according to different
protocols. All lacked t(14;18) translocation. Mutational analysis of
TNFRSF14 gene was performed in 17 cases.
Results: Only six pediatric cases displayed chromosomal imbalances,
with gain/amplification of 6pter-p24.3 (including IRF4) and deletion/
copy number neutral-loss of heterozygosity in 1p36 (including
TNFRSF14) being the most frequent alterations. Sequencing of the
candidate gene TNFRSF14 at 1p36.32 showed nine mutations in seven
cases.
Conclusion: Combination of molecular and genetic features differentiated
a recurrent pattern of genomic imbalances as well as of
TNFRSF14 mutations in pediatric FL which together with other genetic
alterations distinguishes two subsets of pediatric follicular lymphomas.
The first group shows genomic aberrations and is associated with more
aggressive histopathologic and clinical features. The second group lacks
genetic alterations detectable with the present approaches and is
associated with a more limited disease. Despite the absence of genomic
aberrations, these cases resembled FL by their histopathological
features.
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23/04/2013 12:10
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