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

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. M...

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Published in:Journal of neuro-oncology 2021-09, Vol.154 (3), p.327-334
Main Authors: Apra, Caroline, Guillemot, Delphine, Frouin, Eléonore, Bouvier, Corinne, Mokhtari, Karima, Kalamarides, Michel, Pierron, Gaëlle
Format: Article
Language:English
Subjects:
Brain cancer
Clinical Study
DNA-Binding Proteins
Gene expression
Gene set enrichment analysis
Genetics
Hemangiopericytoma - diagnosis
Hemangiopericytoma - genetics
Human health and pathology
Humans
Life Sciences
Medicine
Medicine & Public Health
Meningeal Neoplasms - genetics
Meningioma - genetics
Neurology
Oncology
p53 Protein
Soft Tissue Neoplasms
Soft tissues
Solitary Fibrous Tumors - genetics
Stat6 protein
Transcription Factors
Tumors
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Summary: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.
ISSN:0167-594X
1573-7373
DOI:10.1007/s11060-021-03830-7