ReviewGenes, chromosomes & cancer2020
Clinical and molecular characterization of primary sclerosing epithelioid fibrosarcoma of bone and review of the literature.
Review 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 20 papers.
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Who cites it
20 citing papers in PubMed, 36 citations in OpenAlex.
- Sclerosing epithelioid fibrosarcoma of the bone: a systematic review of radiologic findings and associated clinical features.Annals of medicine and surgery (2012) · 2026Article
- Rare Case of Sclerosing Epithelioid Fibrosarcoma of the Thoracic Spine.Case reports in orthopedics · 2026Article
- Thoracic Spinal Sclerosing Epithelioid Fibrosarcoma Mimicking Schwannoma: Case Report and Literature Review.Current oncology (Toronto, Ont.) · 2025Review
- Article
- A case report from osteosarcoma to SEF: A diagnostic pitfall with a rare metastatic pattern.Radiology case reports · 2025Article
- Article
- Clinicopathological analysis ofWorld journal of clinical cases · 2024Article
- Spontaneous remission of a pulmonary sclerosing epithelioid fibrosarcoma: a case report of a possible abscopal effect.Translational cancer research · 2024Article
- Small cell osteosarcoma versus fusion-driven round cell sarcomas of bone: retrospective clinical, radiological, pathological, and (epi)genetic comparison with clinical implications.Virchows Archiv : an international journal of pathology · 2024Article
- Sclerosing epithelioid fibrosarcoma of bone with hybrid features: clinicopathologic, radiologic, and molecular analysis of three cases.Skeletal radiology · 2024Article
- Report and literature review of four cases of EWSR1::NFATC2 round cell sarcoma.Diagnostic pathology · 2024Review
- Complementary value of molecular analysis to expert review in refining classification of uncommon soft tissue tumors.Genes, chromosomes & cancer · 2024Article
- The Regulatory Network of CREB3L1 and Its Roles in Physiological and Pathological Conditions.International journal of medical sciences · 2024Review
- Response to Immunotherapy in Sclerosing Epithelioid Fibrosarcoma: Case Report and Literature Review.Cureus · 2023Article
- Sclerosing Epithelioid Fibrosarcoma of the Spine: Diagnosis and Treatment of a Rare Entity.Cureus · 2023Article
- First description of hematogenously metastasized sclerosing epithelioid fibrosarcoma arising in the uterine cervix.Gynecologic oncology reports · 2021Article
- The rare primary bone sarcomas: imaging-pathological correlation.Skeletal radiology · 2021Review
- Sclerosing epithelioid fibrosarcoma of bone: morphological, immunophenotypical, and molecular findings of 9 cases.Virchows Archiv : an international journal of pathology · 2021Article
- Case Report: Radiological Features of Sclerosing Epithelioid Fibrosarcoma in the Right Fibula.Frontiers in oncology · 2020Article
- The Use of Crizotinib in Sclerosing Epithelioid Fibrosarcoma withCase reports in oncologyArticle
Corrections and comments
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Authors and funding
10 authors at 5 institutions in 3 countries.
Funding
Abstract
Sclerosing epithelioid fibrosarcoma (SEF) is a rare sarcoma subtype characterized by monomorphic epithelioid cells embedded in a densely sclerotic collagenous matrix. The overwhelming majority of tumors arise in soft tissues; however, rare cases have been documented to occur primarily in bone. The hallmarks of soft tissue SEF include MUC4 immunoreactivity and the presence of an EWSR1-CREB3L1 fusion. Rare cases with alternative fusions have also been reported such as EWSR1-CREB3L2 and FUS-CREB3L2 transcripts. The molecular alterations of skeletal SEF have not been well-defined, with only rare cases analyzed to date. In this study we investigated the clinicopathologic and molecular features of seven patients presenting with primary osseous SEF. There were 3 males and 4 females, with a mean age at diagnosis of 38 years. All cases had microscopic features within the histologic spectrum of SEF and showed strong and diffuse MUC4 positivity, while lacking SATB2 expression. However, due to its unusual presentation within bone, four cases were initially misinterpreted as either osteosarcoma, Ewing sarcoma or chondroblastoma. Half of the patients with follow-up data developed metastasis. The cases were tested by targeted RNA sequencing, MSK-IMPACT, and/or fluorescence in situ hybridization, showing EWSR1-CREB3L1 in six cases and EWSR1-CREB3L2 in one case. The fusion transcripts were composed of EWSR1 exon 11 to either exon 6 of CREB3L1 or CREB3L2. In summary, due to their rarity in the bone, skeletal SEF are often misdiagnosed, resulting in inadequate treatment modalities. Similar to their soft tissue counterpart, bone SEF follow an aggressive clinical behavior and show similar EWSR1-CREB3L1/CREB3L2 fusions.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.