ArticleBrain pathology (Zurich, Switzerland)2018
Intracranial myxoid mesenchymal tumors with EWSR1-CREB family gene fusions: myxoid variant of angiomatoid fibrous histiocytoma or novel entity?
Article in Brain pathology (Zurich, Switzerland), 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 50 papers.
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Who cites it
50 citing papers in PubMed, 87 citations in OpenAlex.
- Pulmonary mesenchymal cystic hamartoma with EWSR1::CREM fusion: molecular redefinition and diagnostic implications.Virchows Archiv : an international journal of pathology · 2026Article
- Spinal EWSR1::CREM fused mesenchymal tumor: an extracranial CNS counterpart of intracranial mesenchymal tumor, FET::CREB‑fusion positive.Brain tumor pathology · 2026Article
- Intracranial mesenchymal tumor, FET::CREB fusion-positive: An integrative analysis of 81 cases.Neuro-oncology · 2026Article
- Identification of a distinct epigenetic subgroup with inferior PFS in intracranial mesenchymal tumors with FET::CREB fusion.Brain pathology (Zurich, Switzerland) · 2026Article
- Rare intracranial mesenchymal tumors with FET::CREB fusions: clinicopathologic spectrum of four cases with integrated molecular analysis and review of the literature.Brain tumor pathology · 2026Article
- Intracranial mesenchymal tumor with FET::CREB fusion: a rare case report.Frontiers in oncology · 2026Article
- Myxoid spindle cell neoplasm with FET::CREB fusion mimicking meningioma: illustrative case.Journal of neurosurgery. Case lessons · 2025Article
- Mesenchymal Nonmeningothelial Tumors of the CNS: Evolving Molecular Landscape and Implications for Neuroradiologists.AJNR. American journal of neuroradiology · 2025Review
- Multidisciplinary treatment of a rare rapidly progressive intracranial myxoid mesenchymal tumor of uncertain differentiation FET-CREB fusion-negative.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Article
- FET-CREB fusion-positive extra-axial myxoid mesenchymal tumor in the cerebellum: illustrative case.Journal of neurosurgery. Case lessons · 2025Article
- EWSR1::CREM rearranged intra-abdominal malignant epithelioid neoplasm: two new cases of an emerging entity with clinicopathological characteristics and histological pitfalls.Virchows Archiv : an international journal of pathology · 2025Article
- A Case of Intracranial Mesenchymal Tumor, FET::CREB Fusion-positive, Diagnosed by Genomic Profiling with FoundationOne CDx.NMC case report journal · 2025Article
- Diagnostic Utility of EWSR1 in Clear Cell Odontogenic Carcinoma: A Systematic Review.Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India · 2024Article
- Primary pulmonary myxoid sarcoma with EWSR1::CREB1 fusion: a literature review.Journal of cancer research and clinical oncology · 2024Review
- Potential prognostic determinants for FET::CREB fusion-positive intracranial mesenchymal tumor.Acta neuropathologica communications · 2024Review
- Daily management of gliomas, glioneuronal, and neuronal tumors in the era of the 2021 WHO classification of nervous tumors.Frontiers in neurology · 2024Article
- Intracranial mesenchymal tumor with (novel) COX14::PTEN rearrangement.Acta neuropathologica communications · 2023Article
- Angiomatoid fibrous histiocytoma: an atypical brain location newly described as intracranial mesenchymal tumor FET-CREB fusion-positive.Acta neurologica Belgica · 2023Article
- Mesenchymal non-meningothelial tumors of the central nervous system: a literature review and diagnostic update of novelties and emerging entities.Acta neuropathologica communications · 2023Review
- Metastatic sporadic paraganglioma with EWSR1::CREM gene fusion: A unique molecular profile that expands the phenotypic diversity of the molecular landscape of the EWSR1::CREM gene fusion positive tumors.Genes, chromosomes & cancer · 2023Article
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Intracranial myxoid mesenchymal tumor harboring EWSR1 fusions with CREB family of genes was recently described, and it resembles the myxoid variant of angiomatoid fibrous histiocytoma. We present three pediatric patients with intracranial EWSR1-rearranged myxoid mesenchymal neoplasm and provide a molecular genetic characterization of these tumors. Clinical histories and imaging results were reviewed. Histology, immunohistochemistry, EWSR1, FUS, NR4A3 fluorescence in situ hybridization (FISH), and next-generation sequencing (NGS) were performed. A 12-year-old male (case 1), 14-year-old female (case 2), and 18-year-old male (case 3), presented with headaches, emesis, and seizures, respectively. The magnetic resonance images demonstrated tumors abutting the dura (cases 1 and 3) and in the third ventricle (case 2). All tumors were vascular, with solid sheets of monomorphic oval cells in a prominent myxoid/microcystic matrix. A thin fibrous pseudocapsule was present in all lesions, but definitive lymphocytic cuffing was absent. Morphologically, they closely resembled myxoid variant of angiomatoid fibrous histiocytoma. Mitoses were rare, and necrosis was absent. All tumors expressed desmin and GLUT1, and focal EMA and CD99. The proliferation index was low. FISH and NGS showed EWSR1-CREB1 fusion (cases 1 and 2), and EWSR1-CREM fusion (case 3). There were no FUS (16p11.2) or NR4A3 (9q22.33) rearrangements in case 3. Gains of 5q (including KCNIP1) and 11q (including CCND1) were present in cases 1 and 2. There were no common pathogenic genomic changes other than EWSR1 rearrangements across cases. CNS myxoid mesenchymal neoplasms with histological and immunophenotypic similarities to myxoid variant of AFH are rare, diagnostically challenging, and harbor EWSR1-CREB1 and also a novel EWSR1-CREM fusion not yet described in AFH. Therefore, it is uncertain if these tumors represent variants of AFH or a new entity. The copy number and mutational changes presented here provide support for future studies to further clarify this issue.
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