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| Optic
nerve gliomas are tumors derived from neuroglial astrocyte elements.
These tumors occur most commonly in children with a median age at
time of diagnosis of five years. Common presenting signs include unilateral proptosis,
decreased acuity, strabismus, papilledema leading to optic atrophy
(#22099),
and occasional CRV occlusion. There
is an increased association with von Recklinghausen's disease or
neurofibromatosis. On gross
pathologic exam, one would see a smooth fusiform enlargement of the optic
nerve(#22101), (#22102). The
tumor often extends into meninges causing meningeal hyperplasia which may
make differentiation from an meningioma difficult on a superficial optic
nerve biopsy. 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(#22106).
Rosenthal fibers are a characteristic but not a diagnostic
degenerative change--fusiform, cigar- shaped, eosinophilic structures
within the astrocyte cytoplasmic processes(#22107).
Some lesions may also show small areas of necrosis within the
tumors which show myxomatous cystic spaces.
-See also
(#28088, #28091, #28094, #28097, #28100) for other views of optic nerve
gliomas.
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| Clinical
#22099 |
Gross
#22101 |
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| High
Power #22107 |
Low
Power #22102 |
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| Med.
Power #22106 |
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