clear cell sarcoma
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Clinical- age: young adults, peak 30-40s
- site: extremities (90%), esp foot/ankle, usually attached to tendons, aponeuroses
- melanocytic differentiation, unrelated to clear cell sarcoma of kidney
Histology- architecture: nests with fibrous septa, fascicles
- cytology: polygonal, spindled, pink or clear cytoplasm, vesicular nuclei, prominent nucleoli
- rare variants: spindle cell, pleomorphic, solid round cell, microcystic, myxoid stroma
StainsDdxMolecular- t(12;22)(q13;q12), EWSR1/ATF1 fusion
- EWS 22q12
- ATF1 12q13
- gastrointestinal variant: EWSR1/CREB1
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