Tumours, masses and neoplasia

Optic nerve glioma

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  • Low-grade astrocytic tumour (technically also a hamartoma)

  • Juvenile (good prognosis) and adult (poor prognosis) forms

  • 50% affect the orbital portion of the nerve, causing

    • Proptosis: axial
    • Optic disc swelling
    • Strabismus
    • Visual loss
  • 50% do not grow but the remainder do and need excision

  • Associated with NF-1 (30-50% of patients)

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Neuroimaging in optic nerve glioma

  • Reveals a fusiform enlargement of the nerve perhaps with kinking within the orbit

Histology

  • Features of pilocytic astrocytomas, Rosenthal fibres (enlarged deeply eosinophilic filaments)

Prognosis

  • Often good but vision may be lost

Treatment

  • For severe proptosis with exposure/cosmesis, intracranial extension or (threatened) chiasmal involvement

  • Surgical excision

  • Radiotherapy

  • Chemotherapy

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