Ain't No Sunshine When You Are Gone!

The initial biopsy of the right optic nerve and sheath demonstrated chronic inflammatory infiltrates and marked fibrocellular changes. The right optic nerve diffusely stained positive for CD45. However there was no gross, microscopic, or immunohistochemical evidence of meningioma or other tumors. Although the initial biopsy was unrevealing, the patient's clinical course and repeated orbital MRI scan supported a diagnosis of infiltrative process. The subsequent procedure was a frontal-orbital craniotomy with unroofing of the right optic canal and right optic nerve resection yielding intracanalicular and intracranial portions of the nerve (see Figure, short edited video will be shown in presentation). 1)Intracanalicular right optic nerve specimen showed dense fibrovascular tissue with macrophage infiltrates. 2)Intracranial portion of the right optic nerve specimen revealed large pleomorphic and hyperchromatic atypical cells infiltrating the nerve. Sectioned tissue underwent immunostaining for GFAP, Ki67, Olig 2, and IDH1. These atypical cells were strongly GFAP positive, but were negative for Olig and IDH1. The Ki67 index of the posterior margin of the tissue was 4-5%. These results were consistent with Anaplastic Astrocytoma WHO Grade III. Malignant Glioma of "Adulthood" is a rare malignant astrocytoma of the anterior visual system that leads to rapidly progressive vision loss, neurological deficits, and poor survival 4. Rapid monocular vision loss, early mimicking of optic neuritis, the development of a retinal artery/vein occlusion, and eventually blindness characterizes this neuro-ophthalmic syndrome1. Malignant gliomas of the optic nerve aggressively spreads to involve the fellow eye in 4-6 weeks, rendering complete blindness, and ultimately, leading to terminal intracranial infiltration3. Malignant gliomas of optic nerve typically occur in middle-aged men1. This case is noteworthy due to the young age of the patient and the undefined clinical course. This patient was managed aggressively with surgical resection of the pre-chiasmal tumor, and treated post-operatively with Temozolomide and stereotactic radiotherapy treatment.