Eye

Orbit & optic nerve

Glioma-optic nerve



Last author update: 1 February 2014
Last staff update: 18 May 2022

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PubMed Search: Glioma optic nerve

Nat Pernick, M.D.
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Cite this page: Pernick N. Glioma-optic nerve. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/eyeorbitgliomaoptic.html. Accessed December 7th, 2022.
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

AFIP images
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MR of large retrobulbar optic nerve tumor

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

AFIP images
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Pilocytic astrocytoma

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Intraparenchymal tumor

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Numerous Rosenthal fibers

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Astrocytes infiltrate meninges

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High grade astrocytoma



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Elongated or hair-like appearance

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Rosenthal fibres

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