ArticleGenes, chromosomes & cancer2015
A genetic dichotomy between pure sclerosing epithelioid fibrosarcoma (SEF) and hybrid SEF/low-grade fibromyxoid sarcoma: a pathologic and molecular study of 18 cases.
Article in Genes, chromosomes & cancer, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 34 papers, 1 of them a synthesis that pooled it.
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Who cites it
34 citing papers in PubMed, 1 synthesis or guideline pooled it, 114 citations in OpenAlex.
- Low-Grade Fibromyxoid Sarcoma of the Distal Upper Extremity: A Systematic Review.Hand (New York, N.Y.) · 2024Pooled it
- Uncommon location, diagnostic challenge: A case series of acral low-grade fibromyxoid sarcoma.Virchows Archiv : an international journal of pathology · 2026Article
- Novel Case of a Molecularly Confirmed Low-Grade Fibromyxoid Sarcoma of the Floor of the Mouth and Literature Review.Head and neck pathology · 2026Review
- Low-Grade Fibromyxoid Sarcoma and Related Subtypes: A Systematic Review and Pooled Analysis of 773 Cases.Cancers · 2026Review
- Rare case report: sclerosing epithelioid fibrosarcoma with FUS-CREB3L1 gene fusion.Frontiers in oncology · 2025Article
- International Multicenter Retrospective Study From the Ultra-rare Sarcoma Working Group on Low-grade Fibromyxoid Sarcoma, Sclerosing Epithelioid Fibrosarcoma, and Hybrid Forms: Outcome of Primary Localized Disease.The American journal of surgical pathology · 2025Article
- A Rare Coexistence of Hybrid Tumor Low-grade Fibromyxoid Sarcoma/Sclerosing Epithelioid Fibrosarcoma and Hibernoma in the Same Thigh: A Case Report.Journal of orthopaedic case reports · 2024Article
- Novel KMT2B gene mutation in MUC4 positive low-grade fibromyxoid sarcoma.Diagnostic pathology · 2024Article
- Sclerosing epithelioid fibrosarcoma of bone with hybrid features: clinicopathologic, radiologic, and molecular analysis of three cases.Skeletal radiology · 2024Article
- An elderly low-grade fibromyxoid sarcoma patient with early postoperative recurrences and metastases: a case report and literature review.Frontiers in medicine · 2024Article
- Response to Immunotherapy in Sclerosing Epithelioid Fibrosarcoma: Case Report and Literature Review.Cureus · 2023Article
- Low-Grade Fibromyxoid Sarcoma in the Hard Palate: A Rare Case Report and Review of the Literature.Journal of maxillofacial and oral surgery · 2023Article
- Sclerosing Epithelioid Fibrosarcoma of the Spine: Diagnosis and Treatment of a Rare Entity.Cureus · 2023Article
- Needle tract seeding of a sclerosing epithelioid fibrosarcoma in a biopsy tract: a case report.BMC musculoskeletal disorders · 2023Article
- The Recent Advances in Molecular Diagnosis of Soft Tissue Tumors.International journal of molecular sciences · 2023Review
- Superficial low-grade fibromyxoid sarcoma.Journal of cutaneous pathology · 2023Article
- Genetic Alterations and Deregulation of Hippo Pathway as a Pathogenetic Mechanism in Bone and Soft Tissue Sarcoma.Cancers · 2022Review
- Low-Grade Fibromyxoid Sarcomas with the Maxillary Sinus Localization: A Case Report and Review of the Literature.Indian journal of otolaryngology and head and neck surgery : official publication of the Association of Otolaryngologists of India · 2022Article
- Low-Grade Fibromyxoid Sarcoma Featuring an UnusualAvicenna journal of medicine · 2022Article
- Establishment and characterization of NCC-LGFMS1-C1: a novel patient-derived cell line of low-grade fibromyxoid sarcoma.Human cell · 2021Article
Corrections and comments
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Authors and funding
7 authors at 1 institution in 1 country.
Funding
Abstract
Sclerosing epithelioid fibrosarcoma (SEF) is a rare soft tissue tumor exhibiting considerable morphologic overlap with low-grade fibromyxoid sarcoma (LGFMS). Moreover, both SEF and LGFMS show MUC4 expression by immunohistochemistry. While the majority of LGFMS cases are characterized by a FUS-CREB3L1 fusion, both FUS-CREB3L2 and EWSR1-CREB3L1 fusions were recently demonstrated in a small number of LGFMS and SEF/LGFMS hybrid tumors. In contrast, recent studies pointed out that SEF harbor frequent EWSR1 rearrangements, with only a minority of cases showing FUS-CREB3L2 fusions. In an effort to further characterize the molecular characteristics of pure SEF and hybrid SEF/LGFMS lesions, we undertook a clinicopathologic, immunohistochemical and genetic analysis of a series of 10 SEF and 8 hybrid SEF/LGFMS tumors. The mortality rate was similar between the two groups, 44% within the pure SEF group and 37% in the hybrid SEF/LGFMS with a mean overall follow-up of 66 months. All but one pure SEF and all hybrid SEF/LGFMS-tested cases showed MUC4 immunoreactivity. The majority (90%) of pure SEF cases showed EWSR1 gene rearrangements by fluorescence in situ hybridization with only one case exhibiting FUS rearrangement. Of the nine EWSR1 positive cases, six cases harbored CREB3L1 break-apart, two had CREB3L2 rearrangement (a previously unreported finding) and one lacked evidence of CREB3L1/2 abnormalities. In contrast, all hybrid SEF/LGFMS tumors exhibited FUS and CREB3L2 rearrangements. These results further demarcate a relative cytogenetic dichotomy between pure SEF, often characterized by EWSR1 rearrangements, and hybrid SEF/LGFMS, harboring FUS-CREB3L2 fusion; the latter group recapitulating the genotype of LGFMS.
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