neuroblastoma


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  • Clinical

    • 3rd most common pediatric malignancy
    • 90% dx by age 5
    • site: abdomen, retroperitoneal, adrenal medulla, ganglia
    • blueberry muffin baby = skin mets
    • urine catecholamines (epi, norepi, HVA, VMA)

  • Histology

    • small round blue cells
    • Homer Wright pseudorosettes
    • can have ganglionic differentiation (ganglioneuroblastoma)

  • Types

    • undifferentiated NB: no gangia, no neuropil, no schwannian stroma
    • poorly differentiated NB: <5% gangia, +neuropil
    • differentiating NB: >5% ganglia, +neuropil, +schwannian stroma
    • nodular GNB: >50% schwannian stroma, macroscopic nests of neuroblastoma
    • intermixed GNB: >50% schwannian stroma microscopic nests of neuroblastoma

  • Stains

    • pos: NSE, chromogranin, synaptophysin, CD56
    • S100 in schwannian stroma

  • Molecular

    • worse prognosis: N-Myc amp, near-diploid, tetraploid, LOH 1p or 11q
    • better prognosis: hyperploidy, TrkA increase

  • Ddx

    • PNET (CD99+)
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