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William Y. Lu, Marc Goldman, Byron Young, and Daron G. Davis

T he incidence of central nervous system (CNS) gangliogliomas ranges from 0.4% to 4.3%. 4, 6, 17, 18 Adolescents and young adults are most commonly affected and gangliogliomas are usually found in the temporal lobe or the floor of the third ventricle. An optic nerve ganglioglioma is extremely rare, and only a few descriptions are included in the literature. 3, 8, 13 We discuss the histological, immunohistochemical, and magnetic resonance (MR) imaging features of this neoplasm. The possible embryological origins are reviewed, and the prognoses of optic nerve

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Optic nerve apoplexy

Report of two cases

Peter L. Reilly and Peter E. Oatey

H emorrhage within the anterior optic pathways is rare. 1 We report the cases of two young women with spontaneous hemorrhage within the optic nerve. The diagnosis of an intraneural cryptic arteriovenous malformation (AVM) was histologically proven in one case and was supported by evidence in the other. Case Reports Case 1 This 31-year-old woman awoke with bilateral retroorbital headache and brief blurring of vision in her left eye. The symptoms improved but returned the following morning accompanied by loss of vision in the left nasal field. During

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W. Craig Clark, Charles S. Theofilos, and James C. Fleming

P rimary optic nerve sheath meningiomas are generally regarded as rare tumors. There has been some degree of confusion regarding the incidence of these tumors because of their infrequent occurrence, the difficulty in determining the actual site of origin in large tumors, 1, 16 and the confusion between meningiomas and the arachnoid hyperplasia that often accompanies optic gliomas. 2 In Cushing's series of 313 meningiomas, 4 there was only one which arose from the sheath of the optic nerve. Other series of both meningiomas and orbital tumors support this

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Nobuhiko Maruoka, Yuzo Yamakawa, and Masaki Shimauchi

I n recent years, intracranial cavernous hemangiomas have been detected more frequently by means of computerized tomography (CT). 8, 9 Although many of these lesions have been reported in intracerebral or extradural locations, a cavernous hemangioma arising in the optic nerve and chiasm is very rare. To our knowledge only two such cases have been reported previously at this location. 2, 3 An additional case with a cavernous hemangioma of the optic nerve is reported; the lesion led to visual impairment of subacute onset after the patient was delivered of a

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Alberto Isla, José Palacios, José M. Roda, Manuel Gutierrez, Cesáreo González, and Martín G. Blázquez

N euroepithelial cysts are rare congenital lesions usually located in the ventricles or cerebral parenchyma. A location for these cysts other than intraventricular and supratentorial is uncommon, and only about 30 such cases have been described in the medical literature. 4, 5, 9, 11 Neuroepithelial cysts arising in the intracranial portion of the optic nerve are also rare, and the few published cases have not been studied histopathologically. 7 We describe a neuroepithelial cyst located within the left optic nerve of a 34-year-old woman. To our knowledge

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Gabib A. Gabibov, Samuil M. Blinkov, and Vasiliy A. Tcherekayev

O ptic nerve gliomas and meningiomas have been subjects of controversy for many years. Optic nerve gliomas grow inside the optic nerve sheath without infiltrating adjacent structures. 8 Primary optic nerve meningiomas arise from the arachnoid sheath of the intraorbital portion of the optic nerve. 9 These neoplasms have been called “optic nerve sheath meningiomas.” Clinically, it is difficult to differentiate an optic nerve glioma from a meningioma in adults. 4, 5 Opinions differ as to the management of optic nerve tumors. The two main approaches to

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Gliomas of the optic nerve or chiasm

Outcome by patients' age, tumor site, and treatment

Ellsworth C. Alvord Jr. and Steven Lofton

O ptic gliomas are primary tumors of the optic nerve and/or chiasm. They generally appear histologically as low-grade astrocytomas, those in the optic nerve invading the leptomeninges and evoking a dense fibroblastic (desmoplastic) reaction and those in the chiasm being rather gelatinous and frequently having an oligodendroglial component. Their natural history has been debated for almost a century, with some investigators 42, 64 believing that they are not neoplasms but hamartomas (that is, that they are congenital malformations and do not grow continuously

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Panagiotis Mastorakos, Michael A. Hays, James P. Caruso, Ching-Jen Chen, Dale Ding, Davis G. Taylor, M. Beatriz Lopes, and Mark E. Shaffrey

M alignant gliomas are the most common and aggressive primary brain tumors. They are most often located in the supratentorial compartment. 29 Malignant optic nerve glioma (MONG) constitutes a rare entity that typically arises in the optic nerve or chiasm but may also present as a part of a multifocal disease process. 37 Patients with MONGs most commonly present with rapidly progressive vision loss, which eventually results in blindness. Similar to malignant gliomas in other locations, these lesions confer a dismal prognosis, with an associated overall survival

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Seiich In, Jun Miyagi, Nobuto Kojho, Shinken Kuramoto, and Masaki Uehara

H emangioblastomas of the brain are most commonly found in the cerebellum; 1, 2, 8 they appear less frequently in the spinal cord and medulla oblongata. 7, 19 A small number of supratentorial lesions have been reported. 4, 5, 9, 14 Lesions of the optic nerve are extremely rare: to our knowledge, only four such cases have been reported previously, in which three tumors were found by chance at autopsy 15, 16, 18 and the fourth was surgically removed. 10 We are now presenting the case of a patient with multiple hemangioblastomas located in the intraorbital

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Robert A. Beatty

C arotid-ophthalmic artery aneurysms, when small, usually project upward, but as they enlarge, there is a tendency for the sac to extend medially, probably because of the restraint provided by the overlying optic nerve. A patient is described in whom a giant carotid-ophthalmic aneurysm was pointing directly upward, splitting the optic nerve. The possible explanations of this unique configuration are discussed. Case Report This 66-year-old right-handed woman presented on February 28, 1985, with an episode of headache, dysphasia, and clumsiness of the