Eye
Orbit and optic nerve
Glioma of optic nerve

Author: Nat Pernick, M.D. (see Authors page)

Revised: 15 May 2018, last major update February 2014

Copyright: (c) 2004-2018, PathologyOutlines.com, Inc.

PubMed Search: Glioma [title] optic nerve

Cite this page: Pernick, N. Orbit and optic nerve: glioma of optic nerve. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/eyeorbitgliomaoptic.html. Accessed September 21st, 2018.
Definition / general
  • Relatively rare
  • Slow growing tumor within orbital segment of optic nerve
  • Usually ages 0 - 9 years with symptoms of minimal exophthalmos, optic nerve atrophy or papilledema
  • Associated with neurofibromatosis type 1
Radiology description
  • Thickening of nerve on CT scan
  • May enlarge optic canal
Radiology images

Images hosted on PathOut server:
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MR - large retrobulbar
optic nerve tumor
causing massive
proptosis

Treatment
  • Resection for tumors limited to optic nerve
  • Also radiation therapy for more extensive lesions
Gross description
  • Small tumors are limited to optic nerve
  • Larger tumors form bulbous enlargement of nerve, often infiltrate pia causing arachnoid thickening
Microscopic (histologic) description
  • Variable cytology and cellularity, even within same tumor, but usually are low grade pilocytic astrocytomas similar to cerebellar and third ventricle tumors with round to spindled nuclei and dendrite-like cytoplasmic processes
  • Often Rosenthal fibers (fusiform, cigar shaped eosinophilic structures within astrocyte cytoplasmic processes, are a nonspecific degenerative change)
  • Rarely are hypercellular with brisk mitotic activity, marked pleomorphism, necrosis and vascular proliferation
  • Difficult to differentiate reactive vs. neoplastic resection margins
  • Typically has intense mucinous degeneration with tumor cells in pools of mucin
  • Infiltrating tumor may cause reactive proliferation of arachnoid cells resembling meningioma
Microscopic (histologic) images

Images hosted on PathOut server:
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Pilocytic astrocytoma
- involves optic nerve
parenchyma and
invades meninges

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Intraparenchymal tumor
with hypercellularlity
and enlargement
of axonal bundles

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Numerous Rosenthal fibers

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Astrocytes infiltrate
meninges, accompanied
by fibroblasts and
meningothelial cells

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High grade astrocytoma
with palisading cells
around blood vessels
and myxoid areas



Images hosted on other servers:
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Elongated or hair-like appearance

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Rosenthal fibres