ArticleVirchows Archiv : an international journal of pathology2021
Sclerosing epithelioid fibrosarcoma of bone: morphological, immunophenotypical, and molecular findings of 9 cases.
Article in Virchows Archiv : an international journal of pathology, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 26 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
- Thoracic Spinal Sclerosing Epithelioid Fibrosarcoma Mimicking Schwannoma: Case Report and Literature Review.Current oncology (Toronto, Ont.) · 2025Review
- Article
- YAP1::KMT2A-rearranged sarcomas harbor a unique methylation profile and are distinct from sclerosing epithelioid fibrosarcoma and low-grade fibromyxoid sarcoma.Virchows Archiv : an international journal of pathology · 2025Article
- Article
- 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
- The Regulatory Network of CREB3L1 and Its Roles in Physiological and Pathological Conditions.International journal of medical sciences · 2024Review
- Infantile Fibrosarcoma of the Bone: A Case Report and Literature Review.Journal of orthopaedic case reports · 2023Article
- Sclerosing Epithelioid Fibrosarcoma of the Spine: Diagnosis and Treatment of a Rare Entity.Cureus · 2023Article
- Sclerosing epithelioid fibrosarcoma of the kidney.BMJ case reports · 2023Article
- Sclerosing epithelioid fibrosarcoma of the foot: A case report.Clinical case reports · 2023Article
- Clinical and pathological studies of eight cases of lipidized fibrous histiocytoma.Experimental and therapeutic medicine · 2023Article
- Metastatic pediatric sclerosing epithelioid fibrosarcoma.Cold Spring Harbor molecular case studies · 2021Review
- Review
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Authors and funding
6 authors at 5 institutions in 4 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Primary sclerosing epithelioid fibrosarcoma (SEF) of bone is a rare and scarcely reported neoplasm. We document clinicopathological and molecular features of 9 additional cases. Five males and 4 females had a mean age of 39 years (14-71 years). Most tumors affected flat/irregular bones; only 3 cases involved a long bone. By radiology, it has characteristic radiographic features of a predominantly lytic expansile lesion with a sclerotic rim. Referring diagnoses were SEF (n = 2), low-grade osteosarcoma (n = 2), chondrosarcoma (n = 1), and chondromyxoid fibroma (n = 1). Histologically, five cases revealed classical morphology of SEF of soft tissue. Remaining cases were classified as hybrid SEF/low-grade fibromyxoid sarcoma, characterized by spindle or stellate cells, prominent stroma, and giant hyalinized areas. Various morphological deviations such as prominent vasculature (n = 3), osteoid-like material (n = 4), or parallel bone trabeculae (n = 2) were observed. Immunohistochemically, all cases showed diffuse and strong MUC4 expression. SATB2 was observed in 5/8 cases. Using FISH, EWSR1, and FUS rearrangements were detected in 4 cases and 1 case, respectively. EWSR1-CREB3L1 fusion was identified in 1 additional case by next-generation sequencing. Recurrence and metastasis were observed in 1 case and 2 cases, respectively. All but one patient were alive with disease for a mean interval of 31 months. SEF of bone is a relatively indolent sarcoma of adults, most commonly located in the flat/irregular bones. Due to overlapping histological features, it is often misdiagnosed as osteosarcoma or a chondroid tumor. Most SEF of bone exhibit EWSR1 rearrangements, but rare cases may harbor a FUS gene fusion.
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