ArticleGenes, chromosomes & cancer2020
Ewing sarcoma with FEV gene rearrangements is a rare subset with predilection for extraskeletal locations and aggressive behavior.
Article in Genes, chromosomes & cancer, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed.
- Clinicopathologic correlates of Ewing (FET::ETS-rearranged) sarcoma and non-Ewing undifferentiated small round cell sarcomas across skeletal and extraskeletal sites.Frontiers in oncology · 2026Article
- Hypoplastic acute myeloid leukemia with aberrant lymphoid antigen expression andTherapeutic advances in hematology · 2026Article
- Rare FUS::FEV-Rearranged Adamantinoma-Like Ewing Sarcoma Arising in the Submandibular Gland.Head and neck pathology · 2025Article
- Small round cell sarcoma tumoroid biobank reveals CIC::DUX4 sarcoma vulnerability to MCL-1 inhibition.Nature communications · 2025Article
- Adamantinoma-like Ewing sarcoma of the parotid gland: A case report.Journal of clinical imaging science · 2025Article
- CRISPR/Cas9 technology in tumor research and drug development application progress and future prospects.Frontiers in pharmacology · 2025Review
- Introduction and impact of routine whole genome sequencing in the diagnosis and management of sarcoma.British journal of cancer · 2024Article
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- Expanding the molecular landscape of undifferentiated sarcomas of bone with a novel EWSR1-SSX3 gene fusion.Genes, chromosomes & cancer · 2024Article
- Soft Tissue Ewing Sarcoma Cell Drug Resistance Revisited: A Systems Biology Approach.International journal of environmental research and public health · 2023Article
- Practical Management of Adult Ultra-Rare Primary Retroperitoneal Soft Tissue Sarcoma: A Focus on Perivascular Epithelioid Tumours and Extraosseous Ewing Sarcoma.Current oncology (Toronto, Ont.) · 2023Review
- Ewing's sarcoma of the duodenum: a rare clinical condition managed with surgical resection.BMJ case reports · 2023Article
- Molecular and biologic biomarkers of Ewing sarcoma: A systematic review.Journal of bone oncology · 2023Review
- Lingual Alveolar Soft Part Sarcoma in a 78-Year-Old Woman: A Case Report and Comprehensive Review of the Literature from 1952 to 2022.Head and neck pathology · 2023Review
- Latest developments in the pathobiology of Ewing sarcoma.Journal of bone oncology · 2022Review
- Review
- Fusion genes as biomarkers in pediatric cancers: A review of the current state and applicability in diagnostics and personalized therapy.Cancer letters · 2021Review
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8 authors.
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Abstract
The molecular hallmark of Ewing sarcoma (ES) is a fusion involving the EWSR1 gene and a member of the ETS family of transcription factors. EWSR1-FLI1 is the most common variant, occurring in 90% of cases, followed by EWSR1-ERG. In a small subset, the FUS gene can substitute for EWSR1 in these fusions. Only rare case reports have been described to date of ES with FEV gene rearrangements. In this study, we investigate the clinicopathologic and molecular features of 10 ES patients with FEV-rearrangements, either fused to EWSR1 (n = 4) or to FUS (n = 6). The median age at diagnosis was 38 years (range, 5-61 years); occurring in six males and four females. All tumors were located at extraskeletal sites, occurring more often in the axial soft tissues. Tumors had a similar morphologic appearance and immunophenotype as ES with more common EWSR1-ETS fusions. Of six patients with follow-up data, five patients (83%) developed metastasis and two patients (33%) died of their diseases. The diagnosis was confirmed either by fluorescence in situ hybridization and/or targeted RNA sequencing. In the five cases tested by targeted sequencing, the fusion transcripts were composed of EWSR1 or FUS fused to either exon 1 or 2 of FEV, retaining the FEV ETS DNA binding domain. This is the largest study to date investigating the ES subset with EWSR1/FUS-FEV fusions showing a predilection for extraskeletal sites and aggressive behavior.
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