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epithelioid sarcoma
Epidemiology:
- most 2nd to 4th decades
- male predominance
Microscopic pathology:
- nodules & clusters of eosinophilic epithelioid or spindled cells
- complex nuclear outlines
- little pleomorphism
- variants:
- large cell or proximal variant: worse prognosis
- angiomatoid variant: contain hemorrhagic cysts lined by epitheliod sarcoma cells
- fibrohistiocytic variant: prominant spindle cells, infiltrated by epithelioid cells
Clinical manifestations:
- fingers, hands, forearm and foot most common
- can occur in trunk, vulva, perineum and inguinal region
- often involves tendons, may ulcerate
- regional lymph node metastases
- distant metastases in 20-30%
- typical sites: lung, soft tissue of scalp
Laboratory:
Immunocytochemistry:
- vimentin: +
- CK8, CK18, CK19: majority of cells +
- CK7: focally + in 20% cases
- CK14: focally +
- CK17: rarely +
- CK20: -
- EMA: +
- CD34: ~50% +, membrane staining
- E-cadherin: -
- V-cadherin: +
- S100: rarely +
- desmin: rarely +
Interactions
disease interactions
General
sarcoma
References
- Miettinen. Diagnostic soft Tissue Pathology.
Churchill Livingstone 2003.