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.
Highlights
IntroductionMeningiomas represent the most common primary intracranial tumor, accounting for 36.6% of newly diagnosed primary brain tumors [17]
Among adult patients, meningiomas represent the most common primary intracranial tumor, accounting for 36.6% of newly diagnosed primary brain tumors [17]
We have performed comprehensive molecular analysis of a large group of pediatric meningiomas, revealing that these tumors are substantially different from their adult counterparts by means of histological subtype distribution, methylation profiles, and mutational landscape
Summary
Meningiomas represent the most common primary intracranial tumor, accounting for 36.6% of newly diagnosed primary brain tumors [17]. Meningiomas are rare among children and young adults, accounting for only 3–5% of intracranial neoplasms [16, 17]. Prior clinical and genetic studies have suggested that differences exist between pediatric and adult meningiomas in regard to anatomical site and clinical behavior [20, 21, 26]. While the vast majority of adult patients with meningiomas. One study showed that pediatric meningiomas harbored NF2 deletions frequently (82%) along with more aggressive histological features (high mitotic count, brain invasion) [21]. It was reported that some pediatric meningiomas may carry YAP1 fusions [35]
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