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Meningeal solitary fibrous tumor cell states phenocopy cerebral vascular development and homeostasis

ABSTRACT: Meningeal solitary fibrous tumor cell states phenocopy cerebral vascular development and homeostasis

PROVIDER: PRJNA988044 | ENA |

REPOSITORIES: ENA

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Meningeal solitary fibrous tumor cell states phenocopy cerebral vascular development and homeostasis

Project description:Meningeal solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms that are associated with hematogenous metastasis, and the cell states and spatial transcriptomic architecture of SFTs are unknown. Here we use single-cell and spatial RNA sequencing to show SFTs are comprised of regionally distinct gene expression programs that resemble cerebral vascular development and homeostasis. Our results shed light on mechanisms underlying SFT biology in comparison to other central nervous system tumors and provide a framework for integrating single-cell and spatial transcriptomic data in human cancer samples.

2025-01-22 | GSE235922 | GEO

Meningeal solitary fibrous tumor cell states phenocopy cerebral vascular development and homeostasis.

Project description: Not available

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Solitary Fibrous Tumours gene profiling study

Project description:Using whole-genome DNA microarrays, we defined the gene expression profiles of 16 Solitary Fibrous Tumours (SFT) and haemangiopericytomas (HPC) (9 HPC and 7 SFT). Expression profiles were compared to publicly available expression profiles of additional SFT or HPC, meningiomas and synovial sarcomas.

2023-02-08 | E-MTAB-11447 | biostudies-arrayexpress

Project description:Meningeal solitary fibrous tumor cell states phenocopy cerebral vascular development and homeostasis

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Gene expression profiling of solitary fibrous tumors

Project description:Sixteen pre-treatment samples of pathologically confirmed solitary fibrous tumors (SFT) were available for RNA profiling. They were collected from 16 patients who underwent initial surgery and/or diagnostic biopsy. Samples were macrodissected by pathologists, and frozen within 30 min of removal in liquid nitrogen in our biobank (Biobank authorization number 2008/70, APHM). All profiled specimens contained more than 70% tumor cells. Each patient gave written informed consent for molecular analysis, and the study was approved by our institutional ethics committee. Total RNA was extracted from frozen samples by using the All-In-One Norgen Biotek kit (Thorold, Canada) and integrity was controlled by Agilent analysis (Bioanalyzer, Palo Alto, CA). Gene expression profiling was done with Affymetrix U133 Plus 2.0 human oligonucleotide microarrays with labeling kit and protocol from manufacturer.

2013-11-12 | E-MTAB-1361 | biostudies-arrayexpress

Identification of BET Inhibitors (BETi) Against Solitary Fibrous Tumor (SFT) Through High-Throughput Screening (HTS)

Project description:Solitary Fibrous Tumor (SFT) is a rare mesenchymal tumor driven by the NAB2-STAT6 fusion oncogene. To investigate transcriptional changes induced by BET inhibition in SFT models, we performed bulk RNA sequencing of INT-SFT and IEC139 cell lines treated with the BET inhibitors Mivebresib (ABBV-075) or BMS-986158 (50 nM for 24 h). Total RNA was extracted and libraries were sequenced on the Illumina NovaSeq X platform. Gene-level expression was quantified using Salmon against the GRCh38 reference genome with GENCODE annotation. Differential expression and pathway enrichment analyses were performed to characterize transcriptional programs associated with BET inhibitor treatment.

2026-03-08 | GSE322765 | GEO

Genomic Sequencing of Solitary Fibrous Tumors

Project description:<p>Solitary fibrous tumors (SFTs) are rare mesenchymal tumors that can be benign or malignant. In this study, we sequenced and analyzed the exomes of 17 SFT matched tumor and normal pairs. This study appertains to a large endeavor to characterize cancers as part of the Slim Initiative for Genomic Medicine in the Americas (SIGMA) project.</p>

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NAB2::STAT6 Fusion Proteins Drive Nuclear Condensate Formation and Transcriptional Reprogramming in Solitary Fibrous Tumors

Project description:Solitary fibrous tumor (SFT) is a rare and aggressive sarcoma driven by NAB2::STAT6 gene fusions, yet effective targeted therapies remain unavailable. Here, we report that the NAB2ex4::STAT6ex2 fusion variant forms nuclear condensates via liquid-liquid phase separation (LLPS) in engineered fibroblast models and primary SFT cells. These condensates co-localize with BRD4S and EGR1, key transcriptional regulators, and are functionally active, driving widespread transcriptional reprogramming. Treatment with Mithramycin A, a compound that disrupts EGR1-DNA interactions, dissolves NAB2::STAT6 condensates and reverses their aberrant gene expression and chromatin binding signatures. Our findings uncover a previously unrecognized role for NAB2::STAT6 in condensate-mediated oncogenic signaling and provide a mechanistic rationale for condensate-targeted therapy in SFT.

2026-06-05 | GSE330326 | GEO

NAB2-STAT6 drives an EGR1-dependent neuroendocrine program in Solitary Fibrous Tumors

Project description:NAB2-STAT6 drives an EGR1-dependent neuroendocrine program in Solitary Fibrous Tumors

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Meningeal cells contribute to postnatal cortical neurogenesis.

Project description:Postnatal brain neurogenesis in mammals is believed to be restricted to rare germinative remnants of the neuroepithelium. In this study, we discovered that, in the postnatal brain, a subset of embryonically derived progenitors is present in meningeal substructures. These cells migrate from the meningeal substructures to the retrosplenial and visual motor cortex and differentiate into (electrically) functional integrated neurons. Lineage tracing analysis revealed that this subset of neural progenitors originate largely from PDGFR+ cells. PDGFR-derived cells differentiate mostly into Satb2+ neurons in cortical layers I-IV. Thus, a reservoir of embryonically derived progenitors in the meninges contributes to postnatal cerebral cortical neurogenesis.

2016-11-23 | E-MTAB-4951 | biostudies-arrayexpress

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