rhabdoid tumor


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  • Clinical

    • 2% of pediatric renal tumors
    • mean age: 1. Rarely over 3.
    • invasive
    • extrarenal sites: CNS, liver, heart, GI, axial

  • Histology

    • architecture: invasive sheets
    • cytology: vesicular chromatin, cherry-red nucleoli, pink cytoplasmic inclusions
    • extensive LVSI
    • frequent mitosis, necrosis
    • can also be predominantly small round blue cells with just focal typical cytology

  • Stains

    • positive: vimentin
    • focal: EMA
    • negative: desmin, myoglobin, CD34

  • Molecular

    • hSNF5/INI1 inactivation, 22q
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