Kirches, Elmar and Sahm, Felix and Korshunov, Andrey and Bluecher, Christina and Waldt, Natalie and Kropf, Siegfried and Schrimpf, Daniel and Sievers, Philipp and Stichel, Damian and Schueller, Ulrich and Schittenhelm, Jens and Riemenschneider, Markus J. and Karajannis, Matthias A. and Perry, Arie and Pietsch, Torsten and Boekhoff, Svenja and Capper, David and Beck, Katja and Paramasivam, Nagarajan and Schlesner, Matthias and Brastianos, Priscilla K. and Mueller, Hermann L. and Pfister, Stefan M. and Mawrin, Christian (2021) Molecular profiling of pediatric meningiomas shows tumor characteristics distinct from adult meningiomas. ACTA NEUROPATHOLOGICA, 142 (5). pp. 873-886. ISSN 0001-6322, 1432-0533
Full text not available from this repository. (Request a copy)Abstract
In contrast to adults, meningiomas are uncommon tumors in childhood and adolescence. Whether adult and pediatric meningiomas differ on a molecular level is unclear. Here we report detailed genomic analyses of 37 pediatric meningiomas by sequencing and DNA methylation profiling. Histologically, the series was dominated by meningioma subtypes with aggressive behavior, with 70% of patients suffering from WHO grade II or III meningiomas. The most frequent cytogenetic aberrations were loss of chromosomes 22 (23/37 [62%]), 1 (9/37 [24%]), 18 (7/37 [19%]), and 14 (5/37 [14%]). Tumors with NF2 alterations exhibited overall increased chromosomal instability. Unsupervised clustering of DNA methylation profiles revealed separation into three groups: designated group 1 composed of clear cell and papillary meningiomas, whereas group 2A comprised predominantly atypical meningiomas and group 2B enriched for rare high-grade subtypes (rhabdoid, chordoid). Meningiomas from NF2 patients clustered exclusively within groups 1 and 2A. When compared with a dataset of 105 adult meningiomas, the pediatric meningiomas largely grouped separately. Targeted panel DNA sequencing of 34 tumors revealed frequent NF2 alterations, while other typical alterations found in adult non-NF2 tumors were absent. These data demonstrate that pediatric meningiomas are characterized by molecular features distinct from adult tumors.
Item Type: | Article |
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Uncontrolled Keywords: | CENTRAL-NERVOUS-SYSTEM; BAP1 MUTATIONS; NF2 GENE; CLASSIFICATION; CHILDREN; GRADE; PROGNOSIS; GERMLINE; FEATURES; SMARCE1; Meningioma; Methylation profile; Targeted sequencing; NF2 |
Subjects: | 600 Technology > 610 Medical sciences Medicine |
Divisions: | Medicine > Abteilung für Neuropathologie |
Depositing User: | Dr. Gernot Deinzer |
Date Deposited: | 30 Sep 2022 12:50 |
Last Modified: | 30 Sep 2022 12:50 |
URI: | https://pred.uni-regensburg.de/id/eprint/48129 |
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