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Neuroblastoma (NB)

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  • Most common solid neoplasm of childhood other than CNS tumors.
  • Traditionally divided into three: NB, ganglioneuroblastoma (GNB) and ganglioneuroma (GN), from most immature to mature; majority are NB.
  • Patients are almost exclusively small children <4 years old.
  • Mostly sporadic or associated with Beckwith-Wiedemann syndrome, neurofibromatosis, and Hirschsprung's disease.
  • May show MYCN amplification, not specific but useful for prognosis.
  • Increased levels of catecholamines and metabolites present.
  • Gross: soft fleshy relatively well-circumscribed mass (image A) & (image B).

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  • Histology:
    • Cells have dark, dusty blue nuclear chromatin and minimal cytoplasm ("small round blue cell" tumor) (image C).
    • Vaguely nodular growth pattern.
    • Homer-Wright rosettes a characteristic feature (seen in only 30%) which consist of a ring-like arrangement of neoplastic cells with a central area of pink neuritic process. No central lumen in the rosettes (image D).
    • Maturation is evidenced by ganglionic differentiation (more eosinophilic cytoplasm, large eccentric nucleus with prominent nucleolus [owl's eye]).
    • Poorly differentiated if <5% ganglion differentiation.
  • Immunohistochemistry: NB84+, neurofilament+, chromogranin+ and synaptophysin+.
  • Prognosis depends on multiple variable (age, histologic features, stage, as well as some molecular (MYCN, DNA ploidy) and genetic (Ch3 1p36, Ch 17q gain).
  • Can metastasize by lymphatics or blood, and commonly to skeletal system especially skull and orbit (Hutchinson's syndrome) and to liver (Pepper's syndrome).
  • DDX: other pediatric small round blue cell tumor such as Ewing sarcoma/primitive neuroectodermal tumor (PNET) (CD99+), rhabdomyosarcoma (desmin+), Wilms' tumor (WT1+) and lymphoma (CD45+).