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Journal of Current Ophthalmology 28 (2016) 228e231http://www.journals.elsevier.com/journal-of-current-ophthalmology

Case report

Optic nerve glioma with complete intraocular extension

Masoomeh Eghtedari a, Seyede Fariba Fattahi a,*, Naser Owji a, Mohammad Javad Ashraf b,Mohammad Reza Khalili a, Bita Geramizadeh c

a Poostchi Eye Research Center, Department of Ophthalmology, Shiraz University of Medical Sciences, Shiraz, Iranb Pathology Department, Khalili Hospital, Shiraz University of Medical Sciences, Shiraz, Iranc Pathology Department, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran

Received 11 April 2016; revised 2 July 2016; accepted 2 July 2016

Available online 3 August 2016

Abstract

Purpose: To present a rare presentation of optic nerve glioma (ONG) with total intraocular extension.Methods: A 44-year-old man with a history of loss of vision since childhood and recent development of progressive disfiguring proptosis wasreferred.Results: The vision in the affected eye was no light perception, and a proptosis of about 2e3 mm was present. Computed tomography andMagnetic Resonance Imaging (MRI) of brain/orbit showed a fusiform enlargement of the left optic nerve and total filling of vitreous cavity by amass with high signal intensity in T2-weighted MRI. There was no extension into the intracranial cavity. The patient also had no signs ofneurofibromatosis. Histopathology confirmed the diagnosis of ONG of the left orbit with extension into the globe and filling vitreous cavity.Conclusion: ONG may extend to the vitreous cavity with no simultaneous intracranial involvement.Copyright © 2016, Iranian Society of Ophthalmology. Production and hosting by Elsevier B.V. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Keywords: Optic nerve; Glioma; Intraocular tumor

Introduction

Optic nerve glioma (ONG) is the most common primarytumor of the optic nerve and is usually detected during the firstdecade of life. The characteristic of ONG is indolent growthwith alternating periods of inactivity and growth with rarecases of spontaneous tumor regression.1 It usually occurs inthe extra-ocular region of the optic nerve.2 ONG arises pre-dominantly between the globe and the orbital apex. The tumorusually remains confined to the orbit or occasionally extendstoward the orbital apex with subsequent involvement of the

No Conflict of interest was declared. No financial support received for this

submission.

* Corresponding author. Poostchi Eye Research Center, Zand St, Shiraz,

Iran.

E-mail address: fariba.fatahi@gmail.com (S.F. Fattahi).

Peer review under responsibility of the Iranian Society of Ophthalmology.

http://dx.doi.org/10.1016/j.joco.2016.07.001

2452-2325/Copyright © 2016, Iranian Society of Ophthalmology. Production and h

license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

chiasm and other parts of brain such as the hypothalamus. Tothe best of our knowledge, only a handful of cases of ONGwith intraocular extension have been reported.3e5

Herein, we present a 44-year-old man with an ONGextended into the globe filling the vitreous cavity.

Case report

The patient was a 44-year-old man with a history of loss ofvision since childhood. He did not seek medical assistanceuntil the age of 44, when he developed proptosis and eyedisfigurement progressively (Fig. 1A). The affected eye had nolight perception. In ophthalmologic examination, the right eyehad no remarkable pathology, but the left eye had about2e3 mm proptosis measured by exophthalmometer. In slitlamp examination, he had whitish opacification of the cornea.Scleral thinning and blue hue from uveal tissue were also seen.However, details of anterior chamber, lens, and posterior

osting by Elsevier B.V. This is an open access article under the CC BY-NC-ND

Fig. 1. A: Left eye disfigurement. B: Orbital CT scan shows fusiform enlargement of optic nerve and tumor occupying vitreous cavity with calcification. C: sagittal

brain MRI T2 weighted. D: Axial post-contrast T1 weighted MRI with fat suppression. C and D show extent of the tumor and absence of intracranial extension.

229M. Eghtedari et al. / Journal of Current Ophthalmology 28 (2016) 228e231

segment were not visible due to severe corneal opacification.Computerized tomography (CT) scan and Magnetic Reso-nance Imaging (MRI) showed a fusiform mass behind theglobe with high intensity signal in T2 weighted images (Fig. 1,BeD) which is characteristic of ONG.3

Enucleation was performed. The globe and retrobulbarmass were excised and underwent histopathological evalua-tion. Gross evaluation of the specimen revealed smooth andfusiform enlargement of the optic nerve as well as a homog-enous solid mass filling almost all of the vitreous cavity(Fig. 2).

Microscopic examination of the Hematoxylin & Eosinslides of the tumor under light microscope (Olympus, ModelBX51) showed a coarsely reticulated and myxomatous patternof spindle-shaped cells, some of which contained cytoplasmiceosinophilic structures compatible with Rosenthal fibers(Fig. 3A, B) as well as areas of mucinous material accumu-lation (Fig. 3C) and presence of focal calcification that is asign of long-lasting tumor (Fig. 3D). The tumor was juvenilepilocytic astrocytomas, grade I, according to the World HealthOrganization (WHO) classification. Complete effacement of

Fig. 2. Complete filling of vitreous cavity by tumor.

the optic nerve and vitreous cavity by the tumor was noted(Fig. 2). Furthermore, there was atrophy of iris and absence ofnormal retina which was completely replaced by the tumor,even though the choroidal tissue was present somewhere(Fig. 3D). Immunostaining demonstrated that the tumor cellswere positive for Glial fibrillary acidic protein (GFAP), S100(focal), and vimentin, negative for cytokeratin, HMB 45, andsynaptophysin, and the Ki67 index was low (Fig. 3, EeF).

The patient was evaluated for signs of neurofibromatosissuch as cutaneous pigmented macules (cafe-au-lait spots),neurofibromas, iris (Lisch) nodules, and osseous defectsinvolving the orbit. However, no sign was found. There was nosign of recurrence during 9 months of follow-up examinations,and prosthetic eye was successfully fitted. Written informedconsent was obtained from the patient.

Discussion

Our patient was a neglected case of ONG with intraocularinvolvement. Sporadic optic pathway gliomas (OPGs) typi-cally present before age 8. Onset can also occur in the seconddecade. Presentation of new symptomatic sporadic OPGs inolder adults up to the fifth decade have been reported occa-sionally.6 In contrast, malignant optic gliomas of adulthoodusually presented with sudden acute visual loss may beassociated with neurological symptoms, and it is rare forthem to result in proptosis.1 Our patient had a history of lossof vision since childhood without seeking medical assistanceuntil the age of 44, when he developed progressive proptosisand eye disfigurement. We think this patient may be aneglected childhood benign ONG. However, presentation ofa new benign ONG in an older adult is another possibility.The remarkable feature of our case is the rare completeintraocular involvement of the ONG. ONG presenting with

Fig. 3. Microphotographs of histopathologic and immunohistochemical analysis. (A) Coarsely reticulated and myxomatous pattern of spindle-shaped cells. (H & E

�100). (B) Cytoplasmic eosinophilic structures (Rosenthal fibers). (H & E,�1000). (C) Areas of mucinous material accumulation. (H & E, �200). (D) Choroidal

tissue was present somewhere. (H & E, �100). (E) The tumor was immunoreactive to GFAP. (�100). (F) Ki67 immunoreactivity was <1%. (�100).

230 M. Eghtedari et al. / Journal of Current Ophthalmology 28 (2016) 228e231

intraocular tumor and seeding in a 3-year-old boy withneurofibromatosis has been reported previously.4 ONG with amanifestation of an elevated oval optic disc mass withextension into the retina has also been reported.5 However, asthe authors have demonstrated, no actual invasion of thevitreous by tumor was noted, and the hyaloid membrane wasintact over the tumor.5 Recently, another case of OPG withintraocular optic nerve involvement in a 3-year-old girl withneurofibromatosis was published.3 Our patient is the firstreport in literature of ONG with complete intraocularextension filling almost all the vitreous cavity in a patientwithout neurofibromatosis. In addition, this case is the firstreport of ONG with intraocular extension filling vitreouscavity presenting in an older adult. This case report presentsa patient who lost all useful vision in his eye due to the latereferral for ophthalmological examination. Clinical pre-sentations of ONG are most often vision loss and

exophthalmos,7 which were observed in our case. Since thetumor is often located completely within the muscle cone andtumor progression is slow, other symptoms such as pain,inflammation, or ophthalmoplegia are only reported in pa-tients with very large tumors.7 Tumors may progress towardthe globe or orbital apex or both, and only rarely affectintraocular structures.8,9

Neurofibromatosis-1 (NF1) has been observed in approxi-mately 25% (range: 10e70%) of cases.1 Our patient had nosigns of NF1.

The best treatment plan for patients with ONG is stillcontroversial.10 The complexity of the disease requires indi-vidualized specific interventional strategies.7 In conclusion,ONG should be considered a differential diagnosis in patientswith intraocular mass and proptosis. It may present even as acomplete intraocular tumor filling almost all the vitreouscavity.

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