Optic nerve seeding of atypical meningiomas presenting with subacute visual loss: 2 case reports with genetic characterization

Report of 2 cases

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Meningiomas rarely cause CSF dissemination, and CSF seeding to the optic nerve (ON) is extremely rare. This is the first report of 2 cases of atypical meningioma with subacute visual loss due to ON seeding. The authors present the genetic characteristics of these atypical meningiomas with CSF dissemination. The patient in Case 1 was a 36-year-old woman with a 1.5-cm mass within the left ON, and the patient in Case 2 was a 70-year-old woman with a 0.9-cm mass around the right ON. Both individuals had undergone multiple surgeries for primary lesions and local recurrent lesions. They presented with subacute visual loss, and both tumors were completely resected. The pathological diagnosis was atypical meningioma with high MIB-1 indices and p53-positive cell ratios in each case. Comparative genomic hybridization showed significant chromosomal copy number alterations similar to the results of previous surgeries, confirming that the tumors were disseminated lesions. The present findings suggest that genetic characteristics, such as 1p and 10qcen-23 losses and 17q and 20 gains, shared by the 2 cases might be associated with CSF dissemination of meningiomas.

Abbreviations used in this paper:CGH = comparative genomic hybridization; CNA = copy number alteration; ON = optic nerve.

Article Information

Address correspondence to: Yohei Kitamura, M.D., Department of Neurosurgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan. email: ykita@sc4.so-net.ne.jp.

Please include this information when citing this paper: published online February 22, 2013; DOI: 10.3171/2013.1.JNS121533.

© AANS, except where prohibited by US copyright law.



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    Case 1. A: Gadolinium-enhanced T1-weighted MR image at initial onset showing an enhanced mass in the right trigone. B: Gadolinium-enhanced T1-weighted MR image of the local recurrence. C: Visual field examination showing no left-field light sense and extreme narrowing of right visual field. D and E: Gadolinium-enhanced T1-weighted MR images acquired after visual impairment showing a 1.5-cm mass in the optic chiasm.

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    Case 1. Intraoperative microscopic findings showing tumor within optic chiasm (arrow), left internal carotid artery (Lt IC), left anterior cerebral artery A1 segment (Lt A1), and right optic nerve (Rt ON) (A). The tumor was completely resected (arrowhead, B). Photomicrograph showing 5 mitoses in 10 hpf (arrow,C). The MIB-1 index was 15.9% (D). The p53-positive cell ratio was 8.3% (E). Merlin immunohistochemistry was positive (F). Comparative genomic hybridization profiles of the local recurrent tumor (G) and disseminated tumor (H). H & E, bar = 50 μm (C–F). Conf. = confidence.

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    Case 2. Gadolinium-enhanced T1-weighted MR image at initial onset showing an enhanced frontal mass (A). Gadolinium-enhanced T1-weighted MR image demonstrating local recurrent tumor (B). Visual field examination showing extreme narrowing of right visual field (C). Gadolinium-enhanced T1-weighted MR image acquired after visual impairment showing a 0.9-cm mass attached to the right ON and diaphragm (arrowheads, D and E).

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    Case 2. Intraoperative microscopic findings showing a tumor (arrow) compressing the right ON (A). The optic canal (OC) was drilled out, and the ON was released. Postoperative microscopic findings (arrowhead,B). Photomicrograph showing atypical cells with enlarged nuclei and whorl formation, and showing 1 mitosis per 10 hpf (arrow)(C). The MIB-1 index was 18.0% (D). The p53-positive cell ratio was 19.0% (E). Merlin was undetectable (F). Comparative genomic hybridization profiles of the local recurrent tumor (G) and disseminated tumor (H). H & E, bar = 50 μm (C–F).



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