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Optic Nerve Glioma

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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.

 

Clinical #22099 Gross #22101
22099.jpg (54837 bytes) 22101.jpg (43407 bytes)
High Power #22107 Low Power #22102
22107.jpg (87533 bytes) 22102.jpg (255216 bytes)
Med. Power #22106
22106.jpg (228039 bytes)
 
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