Letters to the Editor

Pediatric Optic Nerve Sheath Meningioma


e read with great interest the report by Nabavizadeh et al (1) regarding the rare occurrence and aggressive behavior of optic nerve sheath meningioma in the pediatric population (2,3). We had the opportunity to evaluate a 10-year-old girl with a 2-month history of progressive left proptosis. Brain magnetic resonance image (MRI) demonstrated a left intraorbital mass with thickening of the orbital portion of the optic nerve with intense and heterogeneous contrast enhancement. The provisional diagnosis was optic nerve glioma. Two months later, MRI showed enlargement of the mass with extension to the orbital apex but without evidence of intracranial invasion (Fig. 1). Because of rapid tumor growth, the patient underwent frontotemporal craniotomy for tumor excision. Histopathology of the specimen showed fragments of optic nerve surrounded and infiltrated by round aggregates of cells with eosinophilic cytoplasm,

round-to-oval uniform nuclei with some pseudoinclusions. No necrosis and mitotic figures were found. Immunohistochemical staining revealed diffuse cytoplasmic epithelial membrane antigen expression, confirming a meningothelial origin (Fig. 2). Progesterone receptor, glial fibrillary acidic protein, leukocyte common antigen, and S-100 were negative. Ki-67 labeling index was 6%–7%, and Bcl-2 labeling index was 15%–20%. The final diagnosis was meningothelial meningioma, WHO Grade I, with recommendation of careful follow-up because of the Ki-67 index. No recurrence of disease was documented on MRI 3 months later. Clinical behavior of primary optic nerve sheath meningioma seems more aggressive in pediatric patients than in adults, with rapid visual decline and greater likelihood of intracranial extension and recurrence. Although established

FIG. 1. Magnetic resonance imaging of left optic nerve sheath meningioma in the axial plane. (A) T2, (B) precontrast T1, (C) postcontrast T1.

FIG. 2. Histopathology of neoplasm. A. The optic nerve is infiltrated with aggregates of cells containing cytoplasm and uniform nuclei. Necrosis and mitotic figures are absent (hematoxylin and eosin, ·100). B. Immunohistochemical staining shows tumor cells are positive for epithelial membrane antigen (·200). Letters to the Editor: J Neuro-Ophthalmol 2014; 34: 315-319


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Letters to the Editor guidelines are lacking, reports in the literature of optic nerve sheath meningioma in children support surgical excision (2–5). In our patient, rapid progression documented with MRI and the immunohistochemical pattern of a Grade I meningioma with negative progesteron receptor combined with a strongly positive Bcl-2 immunoreaction and Ki-67 proliferative index .3% indicated aggressive behavior of the neoplasm (6). Distinguishing optic nerve sheath meningioma from optic nerve glioma with MRI early in the clinical course may be difficult with neuroimaging (2,3). Gliomas usually are characterized by fusiform enlargement with kinking and tortuosity, with no calcification and variable contrast enhancement. Optic nerve sheath meningiomas often lead to tubular enlargement with homogeneous texture and smooth or slightly lobulated peripheral margins, presence of calcifications, and homogeneous contrast enhancement (7,8). Our case confirms that pediatric optic nerve sheath meningioma is uncommon, but may grow rapidly, exhibiting more aggressive clinical behavior than its adult counterpart. Serena Beccari, MD Luca Cima, MD Ilaria Posenato, MD Francesco Sala, MD Claudio Ghimenton, MD Matteo Brunelli, MD, PhD Albino Eccher, MD, PhD

Departments of Pathology and Diagnostic (SB, LC, IP, MB) and Neurosciences (FS), Azienda Ospedaliera Universitaria Integrata di Verona, Verona, Italy; and Department of Pathology and Diagnostic (CG, AE), Azienda Ospedaliera Universitaria Integrata di Verona, Ospedale Civile Maggiore, Verona, Italy

The authors report no conflicts of interest.

REFERENCES 1. Nabavizadeh SA, Santi M, Belasco JB, Zimmerman RA. Primary atypical optic nerve sheath meniogioma in a child. J. Neuroophthalmol. 2014;34:173–176. 2. Monteiro ML, Gonçalves AC, Siqueira SA, Gebrim EM. Optic nerve sheath meningioma in the first decade of life: case report and review of the literature. Case Rep Ophthalmol. 2012;3:270–276. 3. Harold Lee HB, Garrity JA, Cameron JD, Strianese D, Bonavolontà G, Patrinely JR. Primary optic nerve sheath meningioma in children. Surv Ophthalmol. 2008;53:543–558. 4. Dutton JJ. Optic nerve sheath meningiomas. Surv Ophthalmol. 1992;37:167–183. 5. Eddleman CS, Liu JK. Optic nerve sheath meningioma: current diagnosis and treatment. Neurosurg Focus. 2007;23:E4. 6. Maiuri F, De Caro MB, Esposito F, Cappabianca P, Strazzullo V, Pettinato G, de Divitiis E. Recurrences of meningiomas: predictive value of pathological features and hormonal and growth factors. J Neurooncol. 2007;82:63–68. 7. Ortiz O, Flores RA. Clinical and radiologic evaluation of optic pathway lesions. Semin Ultrasound CT MR. 1998;19:225–239. 8. Ortiz O, Schochet SS, Kotzan JM, Kostick D. Radiologicpathologic correlation: meningioma of the optic nerve sheath. AJNR Am J Neuroradiol. 1996;17:901–906.

Optic Neuritis in the Setting of NMDA Receptor Encephalitis


e read with great interest the report by Sawanura et al (1) on anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. We evaluated a patient who experienced optic neuritis (ON) with optic disc swelling in the setting of anti-NMDAR encephalitis relapse. No evidence of tumor was detected. A 29-year-old otherwise healthy man experienced acute generalized tonic–clonic seizures preceded by severe daily headaches, acute left-sided weakness, and dysarthria. Systemic work-up was normal including autoantibodies (anti-nuclear, anti-DNA, anti-Ro, anti-La, lupus-like anticoagulant, anticardiolipin, anti-beta2-glycoprotein, and anti-neutrophyl cytoplasmic antibodies) as well as cryoglobulins, complement levels, angiotensin-converting enzyme, rheumatoid factor, ammonia and lactate levels, and Coomb test. Serologies were negative for Borrelia burgdorferi; HIV-1 and 2; hepatitis A, B, and C; venereal disease research laboratory test; chlamydia; and mycoplasma. A cerebrospinal fluid (CSF) study revealed lymphocytic pleocytosis with 512 cells per milliliter, elevated


protein of 1.64 g/L, normal glucose, and positive oligoclonal bands. CSF PCR testing for herpes simplex virus, varicellazoster virus, cytomegalovirus, human herpesvirus 6, and enterovirus were all negative. Brain magnetic resonance imaging (MRI) showed a right frontal cortical hyperintensity on T2 and fluid-attenuated inversion recovery (FLAIR) sequences. There was abnormal slow activity in the delta range, predominantly in the right hemisphere on the electroencephalogram (EEG). A diagnosis of presumed lymphocytic meningitis was established. The patient received intravenous methylprednisolone (1 g/day) for 5 days and phenytoin with a clinical improvement. Three months later, brain MRI was unremarkable and the patient was symptom free. Three years later, he was readmitted to hospital because of headache and fluctuating motor and sensory deficits on the left side. Visual function was normal, but there was mild weakness in the left arm and touch impairment. Brain MRI revealed right frontotemporal hyperintensity without contrast Letters to the Editor: J Neuro-Ophthalmol 2014; 34: 315-319

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Pediatric optic nerve sheath meningioma.

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