ArticleThe American journal of surgical pathology2017
EWSR1 Fusions With CREB Family Transcription Factors Define a Novel Myxoid Mesenchymal Tumor With Predilection for Intracranial Location.
Article in The American journal of surgical pathology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 80 papers.
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Who cites it
80 citing papers in PubMed, 138 citations in OpenAlex.
- Mesenchymal tumors of the central nervous system: illustrative case.Journal of neurosurgery. Case lessons · 2026Article
- 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
- Two rare cases of FET::CREB epithelioid mesenchymal tumors, displaying atypical immunophenotype (myoepithelial-like), unraveled on next-generation sequencing.Virchows Archiv : an international journal of pathology · 2026Article
- Intracranial mesenchymal tumor, FET::CREB fusion-positive: An integrative analysis of 81 cases.Neuro-oncology · 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
- Intracranial Mesenchymal Tumor with FET-CREB Gene Fusion: A Case Report of Late Recurrence.OncoTargets and therapy · 2026Article
- Article
- Durable Response to Pazopanib (Tyrosine Kinase Inhibitor) in a Patient With EWSR1::CREM Gene Fusion Positive Intra-Abdominal Unclassified Epithelioid Sarcoma.Genes, chromosomes & cancer · 2025Review
- Malignant epithelioid tumors with EWSR1::CREB fusion involving the kidney: a report of two cases.Virchows Archiv : an international journal of pathology · 2025Article
- Myxoid spindle cell neoplasm with FET::CREB fusion mimicking meningioma: illustrative case.Journal of neurosurgery. Case lessons · 2025Article
- Angiomatoid fibrous histiocytoma occurring at distal/acral extremity sites: clinicopathological and molecular study of 26 cases highlighting frequent myxoid histology and site-dependent genotypic variation.Virchows Archiv : an international journal of pathology · 2025Article
- Dermatofibrosarcoma protuberans with PDGFD rearrangements: a case series featuring a novel EMILIN1::PDGFD fusion and comprehensive literature review.Virchows Archiv : an international journal of pathology · 2025Review
- 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
- Gastric Epithelioid Mesenchymal Tumor with theSurgical case reports · 2025Article
20 more citing papers are in PubMed but not listed here.
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Authors and funding
7 authors at 3 institutions in 3 countries.
Funding
Abstract
Recurrent gene fusions involving EWSR1 with members of the cAMP response element binding protein (CREB) family (ATF1 and CREB1) have been reported in a diverse group of tumors including angiomatoid fibrous histiocytoma (AFH), soft tissue and gastrointestinal clear cell sarcoma, primary pulmonary myxoid sarcoma, and hyalinizing clear cell carcinoma of salivary gland. We have recently encountered a group of 5 myxoid mesenchymal tumors positive for EWSR1 fusions with one of the CREB family member (ATF1, CREB1, and CREM), with histologic features distinct from any of the previously described pathologic entities. Tumors occurred in children or young adults (12 to 23 y; mean, 18 y), with equal sex distribution. All except 1 were intracranial (intra-axial, 2; meningeal, 2), whereas 1 was perirectal. Histologically, the tumors were well circumscribed, often lobulated, composed of uniform ovoid to round cells, and arranged in cord-like or reticular structures in a myxoid background. All except 1 displayed unique sunburst amianthoid fibers. Immunohistochemically, tumors were positive for epithelial membrane antigen (5/5; 4 focal, 1 diffuse) and desmin (3/5). A novel EWSR1-CREM fusion was identified by RNA sequencing in the perirectal tumor, which was further confirmed by fluorescence in situ hybridization (FISH) and reverse transcription-polymerase chain reaction (RT-PCR). A second case with similar EWSR1-CREM fusion was identified by RT-PCR and FISH in a meningeal tumor. The remaining cases studied by FISH showed the presence of EWSR1-CREB1 fusion in 2 cases and EWSR1-ATF1 in 1. In conclusion, we report a distinct group of myxoid mesenchymal neoplasms occurring in children or young adults with a predilection for intracranial locations. Although the immunoprofile [epithelial membrane antigen (EMA), desmin] and the fusion type raise the possibility of a myxoid AFH, none of the typical histologic findings of AFH were present, suggesting a novel entity.
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