Sclerosing epithelioid fibrosarcoma of the bone: a systematic review of radiologic findings and associated clinical features.

BACKGROUND

Primary bone sclerosing epithelioid fibrosarcoma (SEF) is a rare, yet aggressive, condition associated with considerable mortality and prone to misdiagnosis or delayed diagnosis, making precise radiologic identification essential.

MATERIALS AND

METHODS

The systematic review was conducted in accordance with PRISMA standards. PubMed, Scopus, Web of Science, and Embase were searched through March 2025. The inclusion criteria consisted of English-language human studies with radiologic descriptions of primary bone SEF. Two independent reviewers conducted screening and data extraction. The risk of bias was evaluated utilizing the Joanna Briggs Institute critical appraisal tools for case reports and series.

RESULTS

Out of 190 identified papers, 18 met the inclusion criteria, describing 26 patients. The predominant findings in X-rays and computed tomography scans were lysis and destruction (88.46%), well-defined margins (81.81%), and soft tissue involvement (53.84%). magnetic resonance imaging (MRI) data revealed T1-weighted images exhibiting hypo- to isointense signals, while mixed hyper- and hypointense signals mostly characterized T2 sequences. SEF predominantly affected irregular bones (46.2%), followed by long bones (38.5%), with sclerotic rims being most prevalent in long bones (60%). Bone SEF predominantly manifested as pain alone in 40% of cases, and only 33.3% of the patients were initially classified as SEF. Accurate initial diagnosis of SEF was more frequently reported in studies that performed MRI.

CONCLUSION

This systematic review elucidates the varied imaging features of bone SEF across several bone types. When these characteristics elicit suspicion in primary imaging, it is probably wise to prioritize MRI, and early biopsy contemplated to guarantee a timely and precise diagnosis.