clear cell sarcoma


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  • Clinical

    • 3% of pediatric renal tumor
    • bone metastasis
    • mean age: 3
    • mean size: 11cm
    • location: medulla, unicentric

  • Histology

    • architecture: nests, cords, acinar
    • cytology: epithelioid, spindled, high NC ratio, bland (3% is anaplastic)
    • stroma: myxoid, fibrovascular septa
    • can be palisading, sclerosing
    • subtly infiltrative border (entrapped nephrons)

  • Stains

    • vimentin, BCL2+
    • negative for CD34, S100, desmin, CD99, CK, EMA

  • Ddx

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