Epithelioid sarcoma
Epithelioid Sarcoma
- Clinical presentation: Patients 20–40 years + distal extremities — painless nodules on hand/forearm.
- Classic (proximal-type) more aggressive in the pelvic-axillary region, distal-type more common on extremities.
- Histology / IHC: INI-1 (SMARCB1) loss pathognomonic, Cytokeratin+, EMA+, CD34+ in 50%.
- DD: ulcerated SCC, granuloma annulare (in contrast, usually asymptomatic), necrotic pilomatricoma.
- Therapy: Wide resection + adjuvant radiation therapy; for metastatic disease Tazemetostat (EZH2 inhibitor) since FDA approval 2020.
Practise Epithelioid sarcoma in the app
Flashcards with spaced repetition, exam questions and spot-the-diagnosis on this topic – in DermaFuchs, free of charge.
In the DermaFuchs app: 1 flashcards · 3 clinical images
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