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Infiltration of the Optic Chiasm, Nerve, and Disc by Gliomatosis Cerebri

Traynis, Ilana BS; Singer, Samuel MD; Winterkorn, Jacqueline MD, PhD; Rosenblum, Marc MD; Dinkin, Marc MD

Section Editor(s): McCulley, Timothy J. MD

Journal of Neuro-Ophthalmology: March 2014 - Volume 34 - Issue 1 - p 44–46
doi: 10.1097/WNO.0b013e31827912d7
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Abstract: An 18-year-old man with gliomatosis cerebri (GC) developed tumor infiltration of the optic chiasm and right optic nerve including the optic disc. Although papilledema often is seen with GC, tumor invasion of the optic nerve head is observed.

Department of Ophthalmology (IT, JW, MD), Weill Cornell Medical Center, New York, New York; and the Department of Neurology (SS, MR), Memorial Sloan-Kettering Cancer Center, New York, New York.

Address correspondence to Marc Dinkin, MD, 1305 York Avenue, New York, NY 10065; E-mail:

Disclosures: The authors report no conflicts of interest. Marc Dinkin: Advisory Board for Acorda Therapeutics.

An 18-year-old, previously healthy, male college student presented with generalized tonic–clonic seizures and was found to have an area of abnormal T2 signal without enhancement in the right frontal lobe on magnetic resonance imaging (MRI). He was treated with anticonvulsants for 16 months until he developed severe morning headaches, blurred vision, and diplopia. Examination revealed bilateral papilledema, and the MRI demonstrated expansion of the fluid attenuated inversion recovery (FLAIR) abnormality to occupy most of the right cerebral hemisphere and a part of the left frontal lobe (Fig. 1). Magnetic resonance imaging of the anterior visual pathways was unremarkable. Brain biopsy showed World Health Organization Grade 3 astrocytoma (Fig. 2), and in conjunction with the MRI and clinical findings, gliomatosis cerebri (GC) was diagnosed. The patient underwent subtotal resection of the right frontal tumor, followed by radiotherapy and concurrent temozolomide. Papilledema resolved and the vision remained stable for approximately 20 months, when the patient complained of progressive visual decline in the right eye. Magnetic resonance imaging demonstrated enlargement, with some enhancement of the right optic nerve and chiasm, consistent with infiltration by glioma (Fig. 3).

FIG. 1

FIG. 1

FIG. 2

FIG. 2

FIG. 3

FIG. 3

We first evaluated the patient 12 months after onset of visual loss. Visual acuity was no light perception in the right eye and 20/60 in the left eye. Funduscopic examination of the right eye showed a large white mass within the optic disc, and the left optic disc was pale (Fig. 4). Automated perimetry in the left eye showed temporal visual field loss, and optical coherence tomography of the right optic nerve head showed loss of normal structure with cystic spaces.

FIG. 4

FIG. 4

Over 3 months, the patient’s neurologic condition deteriorated, despite a number of chemotherapeutic regimens. He ultimately died, and an autopsy was not obtained.

First described in 1938 by Nevin (1), the current World Health Organization’s classification of brain tumors defines GC as a diffuse, neoplastic, glial infiltration of the brain involving more than 2 cerebral lobes, with no identified tumor mass and with preservation of the architecture of the surrounding tissues (2). Most often, GC develops in men in the third and fourth decades (3).

Diagnosis of GC can be challenging, as the clinical presentation is variable. Early symptoms include cognitive and personality changes and seizures, followed by signs and symptoms of increased intracranial pressure. Focal neurologic findings may develop within months, but visual field deficits are infrequent. Gliomatosis cerebri often involves the hypothalamus, basal ganglia, and corpus callosum (4).

Magnetic resonance imaging has facilitated the diagnosis of GC, but because of its multifocal nature, this neoplasm may be mistaken for other neurologic disorders, such as multiple sclerosis (5) or progressive multifocal leukoencephalopathy (3). Gliomatosis cerebri is difficult to treat because large tumor volumes increase the morbidity associated with radiation therapy. Response rates to chemotherapy are poor, and median survival has been reported at less than 3 years (6).

Invasion of the optic nerves and chiasm occurs in approximately 10% of GC cases (4). Felsberg et al (7) described the case of a young pregnant woman with GC infiltration of the optic nerves and chiasm whose signs and symptoms were initially attributed to multiple sclerosis.

In our patient, GC originated in the right frontal lobe but progressed to involve the optic chiasm and right optic nerve, including the optic disc. We are unaware of similar reports of optic disc infiltration in patients with GC, although a similar optic disc appearance has been described in a patient with optic nerve glioma (8). Early recognition of GC may allow for palliative preservation of vision with focal radiotherapy to the optic apparatus. Additionally, direct visualization of tumor on funduscopy provides a potential opportunity to follow progression of disease or treatment response as an adjunct to neuroimaging.

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© 2014 by North American Neuro-Ophthalmology Society