desmoplastic small round cell tumor


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  • Clinical

    • age: children, young adults
    • site: abdominal cavity, retroperitoneum. Also rarely in paratesticular, thoracic.

  • Histology

    • nests, anastamosing cords
    • uniform small round blue cells
    • desmoplasia in stroma (prominent)
    • frequent mitosis, necrosis

  • Stains

    • positive: WT1, CK, desmin, NSE, EMA, vimentin
    • stroma is positive also for SMA
    • negative: myogenin, myoD1

  • Molecular

    • t(11;22)(p13;q12), EWS/WT1 fusion
    • EWS 22q12
    • WT1 11p13
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