Abstract
Introduction
Meningeal solitary fibrous tumors (SFT), like all SFT, are defined by NAB2–STAT6 fusion and share clinicopathologic similarities with meningiomas, the most frequent meningeal tumors. Our aim is to establish the molecular identity of meningeal SFT and seek molecular prognostic factors.
Methods
RNA sequencing and whole exome sequencing were performed in STAT6-positive SFT and grade 2–3 meningiomas, and data concerning other soft tissues tumors was obtained from the local database. Uniform manifold approximation and projection, individual gene expression and Gene Set Enrichment Analysis were performed.
Results
RNA clustering shows that SFT share a common molecular signature, different from any other type of tumoral tissue. Meningeal SFT aggregate with other SFT, with no clinical or histological subgroup. Comparison of genes expressions suggests significant over-expressions of ZIC2, ZIC3, ZIC5, GABBR2, TP53 in CNS-SFT. The pathogenic TP53 c.743G>T variant, previously undescribed in SFT, was found in one sample of meningeal SFT during malignant progression.
Conclusions
Meningeal SFT are molecular counterparts of extra-meningeal SFT, completely separate from meningiomas. They might develop from the same tissues and benefit from the same treatments as SFT.
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Acknowledgements
The authors thank Genosplice for exome sequencing and Exorare for access to molecular data.
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This work was supported by a grant from the Foundation ARC (PJA 20151203101).
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CA: Conceptualization; Formal analysis; Investigation; Writing—original draft. DG: Formal analysis; Investigation; Writing—original draft. EF: Formal analysis; Investigation. CB: Data curation; Writing—review & editing. KM: Data curation; Writing—review & editing. MK: Conceptualization; Funding acquisition; Supervision; Writing—review & editing. GP: Conceptualization; Methodology; Supervision; Validation; Writing—review & editing.
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Supplementary Fig. 1
Supervised Pearson clustering for SFT and meningiomas samples with clinical data (histological grade, localization of the tumor, sex, age) with 5% genes InterQuantile Range (IQR). List of 5% IQR genes are shown in the table S1 (fichier txt). Supplementary file1 (PNG 230 kb)
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Apra, C., Guillemot, D., Frouin, E. et al. Molecular description of meningeal solitary fibrous tumors/hemangiopericytomas compared to meningiomas: two completely separate entities. J Neurooncol 154, 327–334 (2021). https://doi.org/10.1007/s11060-021-03830-7
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DOI: https://doi.org/10.1007/s11060-021-03830-7