Ophthalmologists may wish to take a biopsy from the optic nerve if the vision is decreasing rapidly or the eye is blind, and there is an urgency to determine if the lesion is inflammatory or neoplastic. Sarcoid of the optic nerve may be associated with sarcoid of the central nervous system: the diagnosis of central nervous system sarcoid may provoke aggressive therapy.
The optic nerve is a tract of the central nervous system, and as such, the tumors arising from the optic nerve are central nervous system tumors (mainly optic nerve gliomas and meningiomas).
It is important that the ophthalmologist performing the biopsy communicate to the pathologist the exact location of the biopsy sample.
- In general, optic nerve gliomas are not tumors that spread beyond the dura (they remain intradural).
- Optic nerve meningiomas may be either intradural or extradural.
- Optic nerve gliomas in children who have neurofibromatosis may comingle with surrounding arachnoidal tissue, stimulating an arachnoidal hyperplasia that may be indistinguishable from meningioma histologically. However, even the arachnoidal hyperplasia of the optic nerve in neurofibromatosis-associated gliomas remains intradural.
Optic nerve biopsies should be processed as one would process biopsy samples from the central nervous system.
Most optic nerve gliomas are pilocytic astrocytomas. Most primary optic nerve menigniomas are meningothelial meningiomas. The pathologist should try to understand from the surgeon the location of the biopsy sample (extradural vs. intradural) before making the diagnosis of optic nerve meningioma in a child. Gliomas of the optic nerve in children with neurofibromatosis may be associated with meningeal hyperplasia that may be histologically indistinguishable from meningioma.
Pilocytic astrocytoma of the optic nerve in a child
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