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OPTIC NERVE GLIOMA
[read also neurofibromatosis on neuropediatry
 Most gliomas of the optic nerve and chiasm (where the two optic nerves come together and separate again inside the brain) are discovered before the age of 10 years.
 Surgery is the treatment of choice for gliomas of the optic nerve (as opposed to the chiasm), particularly when there is profound visual loss or when there is significant protrusion of eyeballs (proptosis).
 Radiation therapy for treatment of optic nerve and chiasmal gliomas remains controversial.
 Some radiation oncologists advocate deferring radiation in patients with no symptoms or in those with lesser visual disturbance until there are signs of disease progression
 In general, tumors of the optic nerve (intracranial as well as intraorbital) have an excellent prognosis following complete surgical excision and/or irradiation
optic nerve which is also known as juvenile pilocytic astrocytoma.
 Optic nerve glioma in adults is a glioblastoma
 ONG is the most common cause of optic nerve enlargement, it accounts for 80% of optic nerve tumors, 1% of all intracranial tumors, and 2% of childhood intraorbital masses.
 80% are in children under 10 years old, and the peak age is 5-8. 90% of patients are under the age of 20. It is more common in females, and 10-50% are in neurofibromatosis patients, especially if bilateral. 15% of NF patients have an optic glioma.
Course
 ONGs grow very slowly and have similar pathology as juvenile pilocytic astrocytomas of the cerebellum. They typically begin intraorbitally and grow in a fusiform shape along the optic nerve toward the globe or toward the brain. Only 25% are confined to the orbit at the time of diagnosis. Malignant degeneration is very rare in children, but in adults, most die within a year of diagnosis. If the tumor begins in the chiasm, it is likely to invade surrounding parenchyma regardless of age. ONGs may extend posteriorly to involve the chiasm, the optic tracts and the optic radiations.
 They often stabilize after childhood.
Clinical Presentation:
Symptoms are :
 proptosis,
 decreased visual acuity,
 exophthalmus,
 optic atrophy,
 papilledema,
 nystagmus,
 increased intracranial pressure,
 and visual field defects.
Disturbances in hypothalamic function such as precocious puberty, increased growth, panhypopituitarism, and diabetes insipidus can occur with large chiasmatic lesions.
Fifty percent of patients are less than 10 years old,
 and 90% present by 20 years old it is associated with neurofibromatosis in 10-20% of cases.
 F = M child / young adult (75% < 10 yrs, 90% < 20 yrs)
Etiology/Pathophysiology:
 Arise from
 optic nerve s,
 optic chiasm,
 and hypothalamus.
 Is slow growing and low grade.
Imaging Findings:
 Fusiform expansion of the optic nerve is seen.
 Lesions in the hypothalamus and optic chiasm grow into the third ventricle
 isodense mass +/- enhancement rarely calcified
 60-70% extend along the optic tract and are therefore not resectable. They rarely calcify, hemorrhage or become cystic.
 On MR, there is usually mild contrast enhancement. The affected optic nerve should be greater than 3 mm in diameter, or 1 mm wider than the unaffected side. It should be hypo- to isointense to muscle on T1 and hyperintense on T2. Fat supression sequences should be performed to see the entire extent of the lesion since it may appear to be more extensive than it is on T2-weighted images secondary to edema.
 Classically, there is a downward kink of the affected optic nerve a few mm posterior to the globe. This may be secondary to increased mucin content, or from nerve elongation. The optic canal is typically widened, and if there is intracranial extension, there should be a low signal lobulated mass. If the mass is large, it will often have some cystic areas and some enhancing solid regions.
Histopathologically,
 most optic nerve gliomas are Grade I or juvenile pilocytic (hair-like) astrocytomas.
 These tumors consist of benign-appearing, round to spindle-shaped astrocytic nuclei with dendrite-like cytoplasmic processes.
 Rosenthal fibers are a characteristic but not a diagnostic degenerative change--fusiform, cigar- shaped, eosinophilic structures within the astrocyte cytoplasmic processes.
 Some lesions may also show small areas of necrosis within the tumors which show myxomatous cystic spaces.
   fusiform enlargement of the optic nerve.
optic nerve meningioma
 female (80%)
 middle age
(24% occur before 10 yrs, but are a/w neurofibromatosis)
 tubular, hyperdense mass
 intense "tramtrack" enhancement
DDX
 Optic nerve sheath meningioma
 Infiltration by leukemia or lymphoma
 Perineural hematoma
 Papilledema of intracranial hypertension
 Patulous subarachnoid space
 Optic neuritis
 Sarcoidosis
Types OF BRAIN TUMORS IN KIDS
Gliomas
Astrocytoma
Ependymoma
Ganglioglioma
Midline Tumors
Hypothalamic-
Chiasmatic (Optic Nerve) Gliomas
Craniopharyngioma
Pineal Region Tumors
Pituitary Tumors
Meningioma
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