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Show Infiltration of the Optic Chiasm, Nerve, and Disc by Gliomatosis Cerebri Ilana Traynis, BS, Samuel Singer, MD, Jacqueline Winterkorn, MD, PhD, Marc Rosenblum, MD, Marc Dinkin, MD 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. Journal of Neuro-Ophthalmology 2014;34:44-46 doi: 10.1097/WNO.0b013e31827912d7 © 2014 by North American Neuro-Ophthalmology Society 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 reso-nance imaging (MRI). He was treated with anticonvulsants for 16 months until he developed severe morning head-aches, blurred vision, and diplopia. Examination revealed bilateral papilledema, and the MRI demonstrated expansion of the fluid attenuated inversion recovery (FLAIR) abnor-mality 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 fron-tal tumor, followed by radiotherapy and concurrent temo-zolomide. Papilledema resolved and the vision remained stable for approximately 20 months, when the patient com-plained of progressive visual decline in the right eye. Mag-netic resonance imaging demonstrated enlargement, with some enhancement of the right optic nerve and chiasm, consistent with infiltration by glioma (Fig. 3). FIG. 1. Axial fluid attenuated inversion recovery (FLAIR) magnetic resonance imaging shows extensive hyperintensity within the right cerebral hemisphere with extension along the corpus callosum to the left hemisphere. From the Department of Ophthalmology (IT, JW, MD), Weill Cornell Medical Center, New York, New York; and the Department of Neu-rology (SS, MR), Memorial Sloan-Kettering Cancer Center, New York, New York. Disclosures: The authors report no conflicts of interest. Marc Dinkin: Advisory Board for Acorda Therapeutics. Address correspondence to Marc Dinkin, MD, 1305 York Avenue, New York, NY 10065; E-mail: mjd2004@med.cornell.edu 44 Traynis et al: J Neuro-Ophthalmol 2014; 34: 44-46 Photo Essay Section Editors: Timothy J. McCulley, MD Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. 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. Over 3 months, the patient's neurologic condition dete-riorated, 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). FIG. 2. Markedly atypical astrocytes permeate the neuro-parenchyma, characteristic of a diffusely infiltrating glioma (hematoxylin and eosin, ·40). FIG. 3. Two years after presentation, magnetic resonance imaging reveals tumor infiltration of the anterior visual pathways. A. Contrasted axial BRAVO image (inversion recovery gradient echo) demonstrates diffuse enlargement and enhancement of the right optic nerve. Contrast-enhanced T1 coronal MRI shows enlargement and mild enhancement of the canalicular right optic nerve (B, arrow) and optic chiasm (C, arrow). FIG. 4. A. There is a massive infiltration of the right optic disc by glioma with extension into the macula. Note the fine vascularity on the surface of the tumor and surrounding subretinal fluid. B. The left optic disc is diffusely pale. Traynis et al: J Neuro-Ophthalmol 2014; 34: 44-46 45 Photo Essay Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. 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 neuro-logic 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 leukoencephal-opathy (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 radio-therapy to the optic apparatus. Additionally, direct visuali-zation of tumor on funduscopy provides a potential opportunity to follow progression of disease or treatment response as an adjunct to neuroimaging. REFERENCES 1. Nevin S. Gliomatosis cerebri. Brain. 1938;61:170-191. 2. Kleihues P, Burger PC, Scheithauer BW. The new WHO classification of brain tumors. Brain Pathol. 1993;3:255-268. 3. Taillibert S, Chodkiewicz C, Laigle-Donadey F, Napolitano M, Cartalat-Carel S, Sanson M. Gliomatosis cerebri: a review of 296 cases from the ANOCEF database and the literature. J Neurooncol. 2006;76:201-205. 4. Peretti-Viton P, Brunel H, Chinot O, Daniel C, Barrié M, Bouvier C, Figarella-Branger D, Fuentes S, Dufour H, Grisoli F. Histological and MR correlations in gliomatosis cerebri. J Neurooncol. 2002;59:249-259. 5. Gottesman M, Laufer H, Patel M. Gliomatosis cerebri: a case report. Clin Neuropathol. 1991;10:303-305. 6. Sanson M, Cartalat-Carel S, Taillibert S, Napolitano M, Djafari L, Cougnard J, Gervais H, Laigle F, Carpentier A, Mokhtari K, Taillandier L, Chinot O, Duffau H, Honnorat J, Hoang-Xuan K, Delattre JY; ANOCEF Group. Initial chemotherapy in gliomatosis cerebri. Neurology. 2004;63:270-275. 7. Felsberg GJ, Glass JP, Tien RD, McLendon R. Gliomatosis cerebri presenting with optic nerve involvement: MRI. Neuroradiology. 1996;38:774-777. 8. Foos RY, Straatsma BR, Allen RA. Astrocytoma of the optic nerve head. Arch Ophthalmol. 1965;74:319-326. 46 Traynis et al: J Neuro-Ophthalmol 2014; 34: 44-46 Photo Essay Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. |
