Optic nerve glioma: A great mimicker

Journal of Clinical Neuroscience, 2010

a b s t r a c t Primary optic nerve sheath meningiomas (ONM) are rare. Most of these are World Health Organization Grade I meningiomas. Because of the intimate relationship to the optic nerve sheath from which they arise, radical excision is often not feasible. The chordoid variant of meningioma is an infrequent tumor and extremely uncommon among primary ONMs. We report a 36-year-old woman with painless proptosis and normal visual acuity who presented to us with an exophytic intraconal mass, which was excised. Histology revealed a chordoid meningioma. We could find only two previous reports, which are discussed.

IntechOpen, 2018

The optic nerve, also known as the second cranial nerve, is composed of axons that transmit visual information from the neurosensory retina to the visual cortex. There are multiple pathologies that can affect the human optic nerve. Congenital anomalies of the optic nerve include myelinated nerve fibers, morning glory syndrome, optic nerve choristoma, optic nerve coloboma, optic nerve hypoplasia and aplasia, and others. Tumors that can affect the optic nerve (ON) may occur primarily from within the nerve itself, from the surrounding optic nerve sheath (ONS), or secondarily spreading to the nerve from a distant site. They include optic pathway glioma, medulloepithelioma, oligodendroglioma, optic nerve sheath meningioma, and others. Here in this chapter, we will review the optic nerve anatomy, embryology, and physiology in addition to assessment of optic nerve function. Moreover, the clinical features, imaging findings, pathology, and treatment options of the most common and some rare congenital anomalies and primary tumors of the ON and sheath will be reviewed.

American Journal of Ophthalmology, 1980

Malignant gliomas of the optic nerve pathways are rare in adults,1.2 and differ from benign optic gliomas that occur in childhood and are considered by some to be hamartomas with self-limiting growth and morbidity.F':" Adult optic glioblastomas are aggressive, causing rapid visual deterioration and subsequent death. They are rarely diagnosed before craniotomy or autopsy.1.2.5 Hoyt and associates 1 examined the clinical and pathologic nature of this tumor and defined a characteristic syndrome consisting of the following: (1) occurrence in middle-aged men; (2) initial signs and symptoms resembling optic neuritis; (3) blindness in two to four months; (4) death in six to nine months. Recent reports indicate that this tumor can occur with visual loss in older individuals of either sex despite normal ophthalmologic and neuroradiologic results.v" We describe herein five cases of malignant optic pathway gliomas, from a single institution, all diagnosed before death. We compared the clinical courses and pathologic features with those in other series. MATERIAL AND METHODS Biopsy specimens from four cases were examined. In three patients (Cases 2-4),

Neuro-Ophthalmology, 2020

Optic nerve choristomas are very rare lesions. They are characterised by the presence of fat cells, mesodermal collection of fibrous tissue and smooth muscle, and atrophic optic nerve tissue. Although the condition can be diagnosed on histology, it is not always possible to carry out surgical exploration for tissue diagnosis in view of the apical location of the lesion and the inherent risk to vision from surgery. Detailed neuro-imaging becomes vital in these cases for diagnosis. We report a case of a patient with an optic nerve choristoma diagnosed on neuroimaging, review the current literature related to this condition, and discuss the key clinico-radiological features of optic nerve choristomas.

British Journal of Neurosurgery, 2009

AJNR. American journal of neuroradiology, 1996

Medical radiology, 2003

Most optic pathway glioma or hypothalamic gliomas are juvenile pilocytic astrocytomas, but their imaging characteristics are not specific with regard to their histologic features. Varying degrees of cystic change and enhancement are demonstrated. The tumours may appear smooth, fusiform, eccentric, or lobulated. Optic pathway gliomas or suprasellar gliomas. These tumours have sometimes been divided into optic pathway gliomas and hypothalamic gliomas (not to be confused with hypothalamic hamartomas). In cases where the tumour is confined to the optic nerves (Dodge stage 1), they can safely be referred to as optic nerve gliomas. Often, however, they are either centred on or extend to involve the chiasm and optic radiations. In such cases, they are difficult to distinguish from hypothalamic gliomas and such a distinction is in most instances artificial. In such more posterior cases the term hypothalamic-optochiasmatic glioma is perhaps more accurate although it certainly does not roll off the tongue. As such, generally, the term optic pathway glioma is favoured, recognising that there may be involvement of the hypothalamus 1). Optic pathway/hypothalamic gliomas (OPHGs) are generally benign but situated in an exquisitely sensitive brain region, and may involve the optic nerves, the optic chiasm, and the optic tracts. Martin and Cushing (1923) first noted the difficulty of determining the site of origin of optic nerve gliomas, since these tend to extend up and down the optic pathways, often making it impossible to decide even at postmortem examination whether the growth originated in the chiasm and extended to the hypothalamus, or vice versa. Epidemiology Gliomas of the optic chiasm and hypothalamus account for 10-15% of supratentorial tumors in children. Males and females are approximately equally affected.

Journal of neuro- …, 2009

Tumors of the optic chiasm are relatively uncommon and usually associated with phakomatoses such as neurofibromatosis. Even more rare is the presentation of a primary, non-exophytic, isolated optic chiasm germ cell tumor (GCT). These tumors have imaging characteristics nearly indistinguishable from optic chiasmatic gliomas (OCGs). Herein we describe two cases of young men who presented with similar findings of progressive, painless visual loss and hypothalamic-pituitary-adrenal axis dysfunction including diabetes insipidus. Brain imaging was non-diagnostic and suggestive of an OCG. Pathology demonstrated GCTs in each case highlighting the importance of biopsy confirmation of the diagnosis. Both patients underwent a pterional craniotomy and sub-frontal approach to the optic chiasm. The chiasm was diffusely enlarged and discolored in each case without evidence of sellar, suprasellar or perichiasmatic pathology. Pathology demonstrated a malignant mixed GCT in the first patient and a germinoma in the second. This case series highlights the importance of tissue biopsy for patients with progressive symptoms from optic chiasm tumors. Furthermore, this is the first report of a primary, non-exophytic malignant mixed GCT. As the treatment regimens differ widely between optic chiasm GCTs and chiasm gliomas, tissue diagnosis is important.

Interdisciplinary Neurosurgery, 2020

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Acta Neuropathologica, 2000

Brat et al. (J Neuropathol Exp Neurol 57 : 288-290, 1998) reported eight cases of a new clinico-pathological entity, which occurs mainly in the third ventricle of middle-aged females, which they described as chordoid glioma of the third ventricle. We report a new case of a 41-year-old woman with a suprasellar chordoid glioma with histological, immunohistochemical and ultrastructural studies. We discuss the differential diagnosis between chordoma, chordoid meningioma, germinoma and pituitary adenoma. Histologically, the tumour showed cords and lobules of isomorphic epithelioid cells in a vacuolated matrix with prominent multifocal lymphoplasmacytic infiltrates in which some histiocytes and isolated Touton-type giant cells were seen; cells were immunoreactive for glial fibrillary acidic protein but negative for epithelial membrane antigen. Ultrastructural examination revealed abundant intermediate filament but no desmosomes, microvilli nor cilia were seen.