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Case report describes diagnostic challenges in bilateral epithelioid sarcoma of the thigh

Case report describes diagnostic challenges in bilateral epithelioid sarcoma of the thigh
Photo by Navy Medicine / Unsplash
Key Takeaway
Consider epithelioid sarcoma in differential for soft-tissue masses with nonspecific imaging.

A case report and systematic literature review describes a 47-year-old woman with bilateral thigh masses. Ultrasonography and magnetic resonance imaging failed to correctly diagnose the tumors. Subsequent histopathological examination demonstrated malignant tumors arising from skeletal muscle, and immunophenotypic findings confirmed a diagnosis of classic-type epithelioid sarcoma. The specific intervention, comparator, follow-up duration, and safety data were not reported. The authors note that epithelioid sarcoma is exceedingly rare, accounting for approximately 1% of all soft-tissue sarcomas. Diagnosis is often delayed because clinical suspicion is low and imaging findings are frequently nonspecific. Key limitations of the evidence include its nature as a single case report, the absence of reported safety or outcome data, and the lack of details on therapeutic strategies. The funding source and potential conflicts of interest were also not reported. For clinical practice, this case underscores that epithelioid sarcoma should remain in the differential for soft-tissue masses, particularly when imaging is inconclusive, and that definitive diagnosis relies on histopathological and immunophenotypic analysis.

Study Details

Study typeSystematic review
EvidenceLevel 1
PublishedMar 2026
View Original Abstract ↓
Epithelioid sarcoma(ES) is an exceedingly rare soft-tissue sarcoma, accounting for approximately 1% of all soft-tissue sarcomas. Despite its typically indolent clinical course, ES carries a substantial risk of local recurrence and distant metastasis, underscoring the importance of early recognition. However, diagnosis is often delayed because clinical suspicion is low and ultrasonography(US)/magnetic resonance imaging(MRI) are frequently nonspecific. In this report, a 47-years-old woman was admitted after incidentally noticing egg-sized masses on the medial aspects of both mid-thighs for one week. Lower Extremity US and Lower Extremity MRI both failed to correctly diagnose the bilateral thigh tumors. Histopathological examination demonstrated malignant tumors arising from skeletal muscle in both thighs. Immunophenotypic findings confirmed a diagnosis of classic-type ES. We further review the literature to summarize the clinical and US/MRI characteristics of ES and to highlight diagnostic pitfalls and current therapeutic strategies.
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