PEComa


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  • Names

    • PEComa
    • perivascular epithelial cell neoplasm
    • sugar tumor

  • Included entities

    • angiomyolipoma
    • lung sugar tumor
    • uterine PEComa
    • lymphangioleiomyomatosis
    • clear cell myomelanocytic tumor
    • other clear cell tumors

  • Histology

    • architecture: perivascular, radial arrangement around lumen
    • cytology: epithelioid (around vessel), spindle (farther from vessel), clear to pink granular cytoplasm
    • usually bland (small central nuclei, small nucleoli), but can be high grade (atypia, mitosis, necrosis)
    • CCMMTs consists almost exclusively of spindle cells in fascicles and nests with vascular septa

  • Stains

    • positive: melanocytic (HMB45, melan-A), muscle (SMA, myosin, calponin)
    • negative: CK, S100
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