Journal of Neuro-Ophthalmology, June 2010, Volume 30, Issue 2

Monocular Nasal Hemianopia From Atypical Sphenoid Wing Meningioma

Neurogenic monocular nasal field defects respecting the vertical midline are quite uncommon. We report a case of a unilateral nasal hemianopia that was caused by compression of the left optic nerve by a sphenoid wing meningioma. Histological examination revealed that the pathology of the meningioma was consistent with that of an atypical meningioma, which carries a guarded prognosis with increased chance of recurrence. The tumor was debulked surgically, and the patient's visual field defect improved.

Monocular Nasal Hemianopia From Atypical Sphenoid Wing Meningioma Rebecca C. Stacy, MD, PhD, Frederick A. Jakobiec, MD, DSc, Simmons Lessell, MD, Dean M. Cestari, MD Abstract: Neurogenic monocular nasal field defects re-specting the vertical midline are quite uncommon. We report a case of a unilateral nasal hemianopia that was caused by compression of the left optic nerve by a sphe-noid wing meningioma. Histological examination revealed that the pathology of the meningioma was consistent with that of an atypical meningioma, which carries a guarded prognosis with increased chance of recurrence. The tumor was debulked surgically, and the patient's visual field defect improved. Journal of Neuro-Ophthalmology 2010;30:160-163 doi: 10.1097/WNO.0b013e3181de8b56 2010 by North American Neuro-Ophthalmology Society FIG. 1. Automated visual fields demonstrate a nasal defect in the left eye. Department of Neuro-Ophthalmology (RCS, SL, DMC); and David G. Cogan Laboratory of Ocular Pathology (RCS, FAJ), Massachusetts Eye and Ear Infirmary, Boston, Massachusetts. None of the authors has any financial conflicts of interest. R. C. Stacy is supported in part by the Heed Foundation. Address correspondence to Rebecca C. Stacy, MD, PhD, Suite 321, 243 Charles Street, Boston, MA 02114; E-mail: Rebecca_Stacy@ meei.harvard.edu 160 Stacy et al: J Neuro-Ophthalmol 2010; 30: 160-163 Photo Essay Copyright © North American Neuro-ophthalmology Society.Unauthorized reproduction of this article is prohibited. A55-year-old woman presented with sudden painless blurred vision of the left eye. The patient had a history of hypertension, diabetes, and hypercholesterolemia, but her ophthalmic and neurologic histories were negative. She was evaluated elsewhere within 2 days of the onset of her symptoms, and nonarteritic anterior ischemic optic neu-ropathy (NAION) was diagnosed. We evaluated her 1 week after the onset of symptoms. The patient's visual acuity was 20/15 in the right eye and 20/25 in the left eye, with dyschromatopsia and a relative afferent pupillary defect in the left eye. No strabismus or motility disturbances were detected, and the left eye had 2 mm of proptosis. Auto-mated perimetry showed a full field in the right eye and a nasal defect in the left eye (Fig. 1). There was temporal pallor of the left optic nerve with no disc edema. Brain MRI with gadolinium revealed a tumor involving the left sphenoid wing measuring 3.3 3 3.6 3 4 cm with extension into the left cavernous sinus and orbital apex, compressing the left optic chiasm and left side of the optic nerve (Fig. 2). The tumor was debulked via a left fronto-temporal craniotomy. Postoperative neuro-ophthalmologic examination showed that the vision was 20/20 in the left eye with a persistent relative afferent pupillary defect. Repeat visual fields showed improvement of the left nasal field defect (Fig. 3). Histopathologic evaluation of the lesion revealed a me-ningioma proliferating in a hypercellular sheet-like fashion with interspersed hemorrhage (Fig. 4A) and foci of fresh necrosis, which lay adjacent to hyalinized fibrosis (Fig. 4B). Some regions manifested the whorl-like morphology of a meningoepithelial meningioma (Fig. 4C), while others contained spindle cells typical of a fibrous meningioma. The nuclei within some of the tumor cells were atypical mani-festing prominent nucleoli (Fig. 4D). The findings of sheeting, hypercellularity, atypical nuclei with prominent nucleoli, and areas of necrosis were consistent with a di-agnosis of atypical meningioma (1,2). A postoperative MRI demonstrated residual tumor abutting the intracranial FIG. 2. Postcontrast T1 axial (A) and coronal (B) MRI show compression of the lateral aspect of the left optic nerve (arrow). FIG. 3. Postoperative left visual field reveals partial res-olution of the nasal field defect. Photo Essay Stacy et al: J Neuro-Ophthalmol 2010; 30: 160-163 161 Copyright © North American Neuro-ophthalmology Society.Unauthorized reproduction of this article is prohibited. portion of the left optic nerve. The patient is currently scheduled for treatment with radiation oncology. Unilateral nasal field defects are extremely rare, and when organic, are often associated with optical phenomena such as subcapsular or traumatic cataract (3,4). Nasal field defects have rarely resulted from aneurysms and mass lesions compressing the temporal side of the optic nerve (5-8). In one case series, nasal field defects due to a dolichoectatic carotid artery, optochiasmatic arachnoiditis, meningioma, or pituitary tumor were associated with optic nerve com-pression and compromised visual acuity (9). In contrast to our patient's defect, in these cases, the superior portions of the nasal fields were often retained. When the meningioma compressing the optic nerve was debulked, the patient's field defect and acuity improved. This is consistent with previous reports. Histological evidence shows that retinal ganglion cell axons that do not cross at the chiasm acquire their tem-poral arrangement in the optic nerve before they reach the optic foramen (10). Therefore, a sphenoid wing menin-gioma or other lesion that compresses the temporal side of the optic nerve would affect the nondecussating fibers resulting in a nasal field defect. With long-standing compression, temporal nerve pallor, as was apparent in our patient, may result. NAION was a diagnostic consider-ation in this patient with a history of sudden vision loss, hypertension, and diabetes. However, the absence of optic disc swelling at the time of presentation made the di-agnosis of NAION unlikely (11). The onset of visual symptoms, which the patient interpreted as sudden, was almost certainly pseudosudden since optic atrophy was al-ready present. FIG. 4. Pathology of the atypical meningioma. A. Area of sheeting with no fascicular or whorled cellular patterns. B. Focus of necrosis (arrows) surrounded by hyalinized fibrous tissue indicating probable healed necrosis. C. Whorl-like pattern common in meningoepithelial tumors. D. The nuclei are atypical and have prominent nucleoli. (Hematoxylin and eosin, (A/B) 3 100; (C) 3 200; and (D) 3 400). Photo Essay 162 Stacy et al: J Neuro-Ophthalmol 2010; 30: 160-163 Copyright © North American Neuro-ophthalmology Society.Unauthorized reproduction of this article is prohibited. Histopathologic review of the patient's lesion revealed an atypical meningioma, which accounts for approximately 15% of meningiomas and are classified as World Health Organization Grade II lesions (12). The sheeting archi-tecture, macronucleoli, and hypercellularity that may reflect a dedifferentiated state can be interspersed with more benign pathologic characteristics (2). Because atypical me-ningiomas have a higher chance for recurrence than the more common benign lesions, approaching 40% as com-pared with 12% for Grade I meningiomas (1), all areas of the biopsied meningioma must be sampled and carefully examined to avoid overlooking an atypical meningioma. Total resection of the lesion can reduce the rate of re-currence and adjuvant fractionated radiotherapy or stereo-tactic radiosurgery may help to control regrowth (13). REFERENCES 1. Perry A, Stafford SL, Scheithauer BW, Suman VJ, Lohse CM. Meningioma grading: an analysis of histologic parameters. Am J Surg Pathol. 1997;21:1455-1465. 2. Perry A, Gutmann DH, Reifenberger G. Molecular pathogenesis of meningiomas. J Neurooncol. 2004;70: 183-202. 3. Rahman I, Nambiar A, Spencer AF. 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Nasal visual field loss with intracranial lesions of the optic nerve pathways. Am J Ophthalmol. 1980;90:1-10. 10. Unso¨ld R, Hoyt WF. Band atrophy of the optic nerve. The histology of temporal hemianopsia. Arch Ophthalmol. 1980; 98:1637-1638. 11. Hayreh SS, Zimmerman MB. Optic disc edema in non-arteritic anterior ischemic optic neuropathy. Graefes Arch Clin Exp Ophthalmol. 2007;245:1107-1121. 12. Louis DN SB, Budka H, von Deimling A, Kepes JJ. Meningiomas. In: Kleihues P, CW, eds. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Nervous System. Lyon, France: IARC Press, 2000:176-184. 13. Modha A, Gutin PH. Diagnosis and treatment of atypical and anaplastic meningiomas: a review. Neurosurgery. 2005;57: 538-550. Photo Essay Stacy et al: J Neuro-Ophthalmol 2010; 30: 160-163 163 Copyright © North American Neuro-ophthalmology Society.Unauthorized reproduction of this article is prohibited.