CNS DLBCL


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  • Clinical

    • < 1% non-Hodgkins
    • 2-3% of brain tumors
    • median age 60
    • site: cerebrum > cerebellum > brainstem
    • tx
      • MTX + ritux if good renal fx
      • non-MTX based chemo if poor renal fx
      • if remission - transplant vs chemo/rad
      • if refractory - salvage chemo

  • Morphology

    • architecture: perivascular, diffuse/solid in denser areas
    • cytology: resembles centroblasts

  • Stains

    • B markers+
    • Mum1 (90%, strong)
    • BCL6 (60-80%)
    • BCL2 (frequent)
    • CD10 (10-20%, if positive consider non-CNS met)

  • Molecular

    • BCL6 rearrangement 17-47%
    • rare MYC rearrangement
    • lack BCL2 rearrangement

  • Ddx

    • metastatic DLBCL
    • immunodeficiency associated
      • EBV+ (PTLD, LyG)
      • HIV associated
    • others
      • intravascular LBCL
      • T/NK, ALCL
      • MALToma in dura
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