Table of Contents
Definition / general | Radiology description | Radiology images | Treatment | Gross description | Microscopic (histologic) description | Microscopic (histologic) imagesCite this page: Pernick N. Glioma-optic nerve. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/eyeorbitgliomaoptic.html. Accessed September 28th, 2023.
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
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