Journal below from

• Bettegowda, C., Chen, L. C., Mehta, V. A., Jallo, G. I., & Rutka, J. T.
(2012). Supratentorial Tumors in the Pediatric Population. Schmidek
and Sweet Operative Neurosurgical Techniques, 669–683.
doi:10.1016/b978-1-4160-6839-6.10056-
Epidemiology
• Optic pathway gliomas are rare tumors accounting for 3-5% of brain tumors in children; 90% are
observed in children. They account for 4% of orbital tumors in children. Association with NF 1 is
classical and the incidence of NF 1 in patients with optic pathway gliomas is estimated at 30-58%.

• https://www.ncbi.nlm.nih.gov/pubmed/20303553

• Optic nerve glioma is a slow-growing tumor, which typically affects children. 30% of patients have
associated NF1 & those have better prognosis.
• Malignant gliomas (glioblastoma) are rare & almost always occur in adult males with a very poor
prognosis & almost certain death within one year. Optic-nerve gliomas Comprise about 1% of all
intracranial tumors.
• About 10% of optic pathway tumors are located within an optic nerve. One third of the tumors involve
both optic nerve and chiasm, a further third involve predominantly the chiasm itself, and one fourth is
predominantly in the hypothalamus. 5 5% gliomas are multicentric
Epidemiology
• Optic nerve gliomas comprise approximately 1% of all intra-cranial tumors, are most commonly
unilateral, and occur more frequently in females.16,17 While they can present atany age, 75%
become symptomatic in the first decade of life and 90% become symptomatic before age 20.18
Rush etal. reported that in their series of 33 patients that the median age at diagnosis was 6.5
years and the mean age was 10.9 years, with a range of 2 to 46 years.19

16. Perkins SM, etal. Glioblastoma in children: a single-institution experi-ence. Int J Radiat Oncol
Biol Phys.
17. Pollack IF, et al. Rarity of PTEN deletions and EGFR amplification in malignant gliomas of
childhood: results from the Children’s Cancer Group 945 cohort. J Neurosurg. 2006;105(suppl
5):418-424.
18. Pytel P. Spectrum of pediatric gliomas: implications for the devel-opment of future therapies.
Expert Rev Anticancer Ther. 2007;7(suppl 12):S51-S60.
19. Ruggiero A, et al. Phase II trial of temozolomide in children with recur-rent high-grade glioma.
J Neurooncol. 2006;77(1):89-94.
Clinical Features
• Visual loss and optic atrophy are the most common signs. Proptosis occurs if the
tumor is in the orbit.
Vaughan, Asbury. General Ophthalmology

• Optic atrophy is a sign and typically is noted as optic nerve pallor. This is the end
stage of a process resulting in optic nerve damage. Because the optic nerve fiber
layer is thinned or absent the disc margins appear sharp and the disc is pale,
probably reflecting absence of small vessels in the disc head.
Symptoms
• The main symptom of optic atrophy is vision loss. Any other symptoms are
attributable to the underlying process that caused the disc damage (such as pain
with angle closure glaucoma)
https://eyewiki.aao.org/Optic_Atrophy
Optic Atrophy
• Ophthalmoscopic optic atrophy
Primary optic atrophy
• In conditions with primary optic atrophy (eg, pituitary tumor, optic nerve tumor, traumatic optic neuropathy, multiple
sclerosis), optic nerve fibers degenerate in an orderly manner and are replaced by columns of glial cells without alteration
in the architecture of the optic nerve head. The disc is chalky white and sharply demarcated, and the retinal vessels are
normal. Lamina cribrosa is well defined.

Secondary optic atrophy

• In conditions with secondary optic atrophy (eg, papilledema, papillitis), the atrophy is secondary to papilledema (shown in
the image below). Optic nerve fibers exhibit marked degeneration, with excessive proliferation of glial tissue. The
architecture is lost, resulting in indistinct margins. The disc is grey or dirty grey, the margins are poorly defined, and the
lamina cribrosa is obscured due to proliferating fibroglial tissue. Hyaline bodies (corpora amylacea) or drusen may be
observed. Peripapillary sheathing of arteries as well as tortuous veins may be observed. On visual fields, progressive
contraction of visual fields may be seen
• https://emedicine.medscape.com/article/1217760-overview#a5
Clinical Features
• in contrast, malignant optic gliomas of adulthood usually pres-ent with sudden acute
visual loss in 70% to 84% of patients19,20 But,unlike optic gliomas of childhood, it is
exceptionally rare for malig-nant gliomas to result in proptosis.22Up to one-third of
patients may also have associated neurological symptoms such as head-aches, eye pain,
hemiplegia, and sometimes dementia.

19.Hartel PH, Rosen C, Larzo C, et al. Malignant optic nerve glioma

(glioblastomamultiforme): a case report and literature review.W V Med
J2006;102:29–31.
20. Wabbels B, Demmler A, Seitz J, et al. Unilateral adult malignant optic
nerveglioma.Graefes Arch Clin Exp.
22. Dinh TT, Wang YY, Rosenfeld JV, et al. Glioblastoma of the optic chiasm.J
ClinNeurosci2007;14:502–5.