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Optic Nerve Glioma




Optic pathway tumors are generally classified according to their location along the optic pathway.


This classification correlates highly with:

  • The appropriate treatment for the disease
  • Symptoms associated with the tumor
  • Patient prognosis


The three major categories are:

    • Optic nerve gliomas
    • Chiasmatic gliomas
    • Chiasmatic/ hypothalamic gliomas 

Optic nerve gliomas can involve one or both optic nerves. 

If a child has bilateral tumors, then it is very likely that the child has NF-1.


Characteristics of Optic Nerve Gliomas:

Optic Nerve Gliomas

Areas involved

  • May be unilateral or bilateral


  • Usually <5 years (when not associated with NF-1)


  • Asymptomatic
  • Proptosis
  • Optic atrophy
  • Impaired visual acuity

Clinical Course

  • Variable, but generally non-progressive

MR scan

Tumor is generally:

  • Small
  • Well circumscribed
  • Homogeneous enhancement with gadolinium


Not necessary for diagnosis

Principles of Therapy

  • Initial close follow up
  • Active intervention at time of progression
  • If there is no remaining vision at the time of presentation, surgical resection recommended
  • To preserve useful vision, chemotherapy can be used for younger children and radiation therapy (RT) for older children

Long-term survival

  • Approaching 100%


Characteristics of Chiasmatic Gliomas:

Chiasmatic Gliomas

Affected areas

  • Optic chiasm
  • One or both optic nerves


  • Loss of visual acuity
  • Temporal field defects

Clinical course

  • At age < 5 years tumor may progress within a few months of diagnosis

MR Scan

Tumor is generally:

  • Small
  • Well circumscribed
  • Homogeneous enhancement with gadolinium


  • Generally unnecessary

Principles of Therapy

  • Period of surveillance after diagnosis
  • Same use of chemotherapy and RT as with optic gliomas when progression is observed

Long-term survival

  • 90-100%


Characteristics of Chiasmatic/Hypothalamic Gliomas:

Chiasmatic/Hypothalamic Gliomas

Affected areas

  • Originate in optic chiasm
  • Extends to hypothalamus
  • May fill the 3rd ventricle


Early symptoms include:

  • Nystagmus
  • Impaired visual acuity
  • Visual field deficits

Late symptoms include:

  • Signs of raised intracranial pressure (hydrocephalus)

Clinical Course


  • Large tumor
  • More aggressive than other varieties




  • Treatment of hydrocephalus with shunt
  • Surgical resection if possible(rarely able to achieve total resection)
  • Post-operative adjuvant therapy (chemotherapy and or radiation therapy for disease progression)

Long-term survival

  • 50-80%


  • No generally accepted staging system for optic pathway gliomas. 
  • Low-grade gliomas and tend to be slow-growing.
  • Large infiltrating lesions may invade the hypothalamus, but distinctions between optic and hypothalamic tumors are not usually of any clinical relevance. 

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