Gliomatosis cerebri in children: A poor prognostic phenotype of diffuse gliomas with a distinct molecular profile
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F65269705%3A_____%2F24%3A00079931" target="_blank" >RIV/65269705:_____/24:00079931 - isvavai.cz</a>
Alternative codes found
RIV/00216224:14110/24:00138853 RIV/00216208:11130/24:10480041 RIV/00064203:_____/24:10480041
Result on the web
<a href="https://academic.oup.com/neuro-oncology/advance-article/doi/10.1093/neuonc/noae080/7667099" target="_blank" >https://academic.oup.com/neuro-oncology/advance-article/doi/10.1093/neuonc/noae080/7667099</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1093/neuonc/noae080" target="_blank" >10.1093/neuonc/noae080</a>
Alternative languages
Result language
angličtina
Original language name
Gliomatosis cerebri in children: A poor prognostic phenotype of diffuse gliomas with a distinct molecular profile
Original language description
BackgroundThe term gliomatosis cerebri (GC), a radiology-defined highly infiltrating diffuse glioma, has been abandoned since molecular GC-associated features could not be established.MethodsWe conducted a multinational retrospective study of 104 children and adolescents with GC providing comprehensive clinical and (epi-)genetic characterization.ResultsMedian overall survival (OS) was 15.5 months (interquartile range, 10.9-27.7) with a 2-year survival rate of 28%. Histopathological grading correlated significantly with median OS: CNS WHO grade II: 47.8 months (25.2-55.7); grade III: 15.9 months (11.4-26.3); grade IV: 10.4 months (8.8-14.4). By DNA methylation profiling (n = 49), most tumors were classified as pediatric-type diffuse high-grade glioma (pedHGG), H3-/IDH-wild-type (n = 31/49, 63.3%) with enriched subclasses pedHGG_RTK2 (n = 19), pedHGG_A/B (n = 6), and pedHGG_MYCN (n = 5), but only one pedHGG_RTK1 case. Within the pedHGG, H3-/IDH-wild-type subgroup, recurrent alterations in EGFR (n = 10) and BCOR (n = 9) were identified. Additionally, we observed structural aberrations in chromosome 6 in 16/49 tumors (32.7%) across tumor types. In the pedHGG, H3-/IDH-wild-type subgroup TP53 alterations had a significant negative effect on OS.ConclusionsContrary to previous studies, our representative pediatric GC study provides evidence that GC has a strong predilection to arise on the background of specific molecular features (especially pedHGG_RTK2, pedHGG_A/B, EGFR and BCOR mutations, chromosome 6 rearrangements).
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30204 - Oncology
Result continuities
Project
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Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2024
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Neuro-Oncology
ISSN
1522-8517
e-ISSN
1523-5866
Volume of the periodical
26
Issue of the periodical within the volume
9
Country of publishing house
US - UNITED STATES
Number of pages
15
Pages from-to
1723-1737
UT code for WoS article
001458375800001
EID of the result in the Scopus database
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