retiform hemangioendothelioma

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  • Clinical

    • site: distal extremities (skin, subq)
  • Histology

    • architecture: resembles rete testis - vascular slits, lined by blue tumor cells
    • lining: blue endothelial cells, hobnail appearance, high nc ratio. Lack of pleomorphism or mitosis
    • stroma: sclerotic, 1/2 have lymphocytic infiltrate, can also have solid tumor areas
  • Stains

    • positive: CD31, CD34, VWF
    • lymphocytic infiltrate is predominantly T cells
    • negative: HHV8