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Molecular description of meningeal solitary fibrous tumors/hemangiopericytomas compared to meningiomas: two completely separate entities

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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https://hal-amu.archives-ouvertes.fr/hal-03660728
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Submitted on : Friday, May 6, 2022 - 10:54:48 AM
Last modification on : Tuesday, May 10, 2022 - 3:49:57 AM

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Caroline Apra, Delphine Guillemot, Eléonore Frouin, Corinne Bouvier, Karima Mokhtari, et al.. Molecular description of meningeal solitary fibrous tumors/hemangiopericytomas compared to meningiomas: two completely separate entities. Journal of Neuro-Oncology, Springer Verlag, 2021, 154 (3), pp.327-334. ⟨10.1007/s11060-021-03830-7⟩. ⟨hal-03660728⟩

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