Primary tumours of the optic nerve and its sheath

Miller, Neil R.

doi:10.1038/sj.eye.6701592

Cited by 193 publications

(171 citation statements)

References 40 publications

(45 reference statements)

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“…Neuroradiologic findings are unspecific, usually described as contrast enhancement and eventual thickening of the optic nerve, chiasm or tract in T1-weighted images [4,26], with iso-to hypointensity on native T1 images [27,28]. T2 hyperintensity of the affected anterior visual pathway is a matter of debate [27].…”

Section: Resultsmentioning

confidence: 99%

Malignant optic glioma – the spectrum of disease in a case series

Traber

Pangalu

Neumann

et al. 2015

Graefes Arch Clin Exp Ophthalmol

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PURPOSE Malignant optic glioma of adulthood is a rare, invasive neoplasm of the anterior visual pathway with 66 cases reported in the literature. It presents as anaplastic astrocytoma (WHO grade III) or glioblastoma (WHO grade IV). The present case series covers the spectrum of disease manifestations, discusses neuroradiological findings, and reviews the current literature. METHODS Retrospective case series of five patients from three tertiary referral centers and literature review. RESULTS Visual loss with or without pain was the presenting symptom in all patients (two women, three men). Two patients were initially misdiagnosed as optic neuritis, and one patient as atypical non-arteritic anterior ischemic optic neuropathy (NAION). A neoplastic disease was suspected in the two remaining patients. MRI features were iso-to hypointensity on T1-weighted native images, contrast enhancement, and hyperintensity on T2-weighted images. Biopsy was generally diagnostic; however, one patient required two biopsies for diagnosis. The series includes an exceptional case of intraocular tumor extension and vitreous spread. The disease was lethal within one to two years in all patients. CONCLUSIONS Malignant optic glioma is a diagnostic challenge and remains a devastating and lethal disease. Advances in the understanding of tumor biology have yet failed to translate into effective treatment regimens. ResultsVisual loss with or without pain was the presenting symptom in all patients (two women, three men). Two patients were initially misdiagnosed as optic neuritis, and one patient as atypical non-arteritic anterior ischemic optic neuropathy (NAION). A neoplastic disease was suspected in the two remaining patients. MRI features were iso-to hypointensity on T1-weighted native images, contrast enhancement, and hyperintensity on T2-weighted images. Biopsy was generally diagnostic, however, one patient required two biopsies for diagnosis. The series includes an exceptional case of intraocular tumor extension and vitreous spread. The disease was lethal within one to two years in all patients. Conclusions Powered by Editorial Manager® and ProduXion Manager® from Aries Systems CorporationMalignant optic glioma is a diagnostic challenge and remains a devastating and lethal disease. Advances in the understanding of tumor biology have yet failed to translate into effective treatment regimens. Powered by Editorial Manager® and ProduXion Manager® from Aries Systems CorporationCover Letter Click here to download Cover Letter: revised Graefes MOG Cover Letter.doc Response to referees' commentsThe thorough review of our manuscript was much appreciated. We have addressed the issues raised and suggestions made by the reviewers.Reviewer comments:Reviewer #1: The authors report on 5 patients with malignant optic gliomas. The text is concise and very informative with excellent illustrations. For the sake of completeness the authors may wish to add some minor data to the text. COMMENT 1. A simplified table showing the current WHO classifica...

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Section: Resultsmentioning

confidence: 99%

Malignant optic glioma – the spectrum of disease in a case series

Traber

Pangalu

Neumann

et al. 2015

Graefes Arch Clin Exp Ophthalmol

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“…3,8,13) All patients with optic nerve hemangioblastoma suffer progressive visual disturbance leading to blindness, so total removal in the early stage is recommended. 8,10,13) Prompt recognition of this rare entity is important because early diagnosis and treatment can preserve the vision.…”

Section: Discussionmentioning

confidence: 99%

Hemangioblastoma of the Optic Nerve-Case Report-

Higashida

Sakata

Kanno

et al. 2007

Neurol. Med. Chir.(Tokyo)

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A 64-year-old man presented with a rare sporadic hemangioblastoma arising in the left optic nerve manifesting as left visual disturbance gradually progressive over 5 years. Magnetic resonance imaging revealed a well-enhanced mass in the left optic nerve. Partial resection of the tumor was performed via the frontoorbital approach. The histological diagnosis was optic nerve hemangioblastoma. Hemangioblastoma must be considered in the differential diagnosis of optic nerve tumors even in the absence of other lesions associated with von Hippel-Lindau disease.

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“…Common findings in patients with this disease are visual loss, optic atrophy and optociliary shunts (considered the pathognomonic triad). Furthermore, proptosis and disc swelling are often seen (Miller 2004). Diffuse tubular enlargement is commonly seen on computed tomography (CT) scans and a tram-tracking sign characteristic of meningioma may be seen with magnetic resonance imaging (MRI) techniques (Lindblom et al 1992).…”

Section: Optic Nerve Sheath Meningiomamentioning

confidence: 99%

“…Primary tumours of the optic nerve are typically benign and consist mainly of glioma and meningioma, although primary malignant tumours do exist (Eggers et al 1976;Spoor et al 1980;Miller 2004). Retinoblastoma and uveal melanoma are the most frequent secondary tumours diagnosed in the optic nerve, but leukaemia and lymphoma of the eye region are becoming more frequent because therapeutic advances lead to longer survival periods in these patients, and thus to more metastases (Christmas et al 1991;Sharma et al 2004).…”

Section: Tumours Of the Optic Nervementioning

confidence: 99%

Primary and secondary tumours of the optic nerve, with emphasis on invasion by uveal malignant melanoma

Lindegaard

2007

Acta Ophthalmologica Scandinavica

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Tumours of the optic nerve may originate in the optic nerve itself (i.e. primary tumours) or in structures adjacent to the optic nerve (i.e. secondary tumours). Furthermore, leukaemic infiltration and tumour metastases from distant solid tumours are also found in the optic nerve (i.e. secondary tumours). Large surveys have described primary optic nerve tumours and their treatment modalities, whereas secondary tumours have received little attention. Most reported work-ups have been based on material from referral centres and specialized departments.The correlation between invasion of the optic nerve in uveal melanoma and the risk of death from the disease has not been fully elucidated. The impact on prognosis may be correlated with tumour cells left in the proximal nerve after enucleation. In addition, melanoma with secondary invasion of the optic nerve may represent a highly malignant invasive subcategory of uveal melanoma. Clinical factors that make the ophthalmologist aware of optic nerve invasion when investigating a uveal melanoma are most important.Melanomas that invade the optic nerve may histopathologically display different invasive patterns and represent different types of uveal melanoma. A subtype of uveal melanoma with a preponderance for neural invasion may exist. Such a subtype has been identified for cutaneous melanoma (desmoplastic ⁄ neurotropic).In order to investigate tumours of the optic nerve in a known population, we studied all optic nerve tumours that had been surgically removed in Denmark over a period of 25 years. Furthermore, in order to resolve questions about secondary invasion of the optic nerve in uveal melanoma, we investigated all eyes with uveal melanoma and optic nerve invasion enucleated in Denmark between 1942 and 2001 (n ¼ 157).Tumours of the optic nerve in children were dominated by optic glioma and invasion from retinoblastoma, whereas tumours in adults mainly comprised optic nerve sheath meningioma and invasion from uveal melanoma.Optic nerve invasion of uveal melanoma was found in one in 20 patients. Increased IOP and juxtapapillary location were associated with both pre-laminar ⁄ laminar and post-laminar invasion of the optic nerve. Furthermore, ageReuse of this article is permitted in accordance with the Creative Commons Deed, Attribution 2.5, which does not permit commercial exploitation. Acta Ophthalmologica Scandinavica 20071 over 70 years, vision reduced to light perception, non-visible fundus and large tumour size were associated with post-laminar invasion. Only patients with optic nerve invasion had metastases to the CNS, kidney and heart. Histopathological features associated with optic nerve invasion were focal retinal invasion, neovascularization of the chamber angle and scleral invasion. Furthermore, non-spindle cell type and rupture of the ILM were all associated with post-laminar invasion.The optic nerve was invaded in four different ways:(1) by tumour extension from the neuroretina through the lamina cribrosa; (2) by direct extension into the optic ...

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Primary tumours of the optic nerve and its sheath

Abstract: Purpose To describe the clinical, neuroimaging, and pathologic features of primary tumours of the optic nerve and its sheath.

Cited by 193 publications

References 40 publications

Malignant optic glioma – the spectrum of disease in a case series

Malignant optic glioma – the spectrum of disease in a case series

Hemangioblastoma of the Optic Nerve-Case Report-

Primary and secondary tumours of the optic nerve, with emphasis on invasion by uveal malignant melanoma

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