Authors: Lindegaard, Jens; Heegaard, Steffen

Source: Expert Review of Ophthalmology, Volume 4, Number 2, April 2009 , pp. 197-206(10)

Publisher: Expert Reviews

Abstract:

A variety of lesions may involve the optic nerve. Mainly, these lesions are inflammatory or vascular lesions that rarely necessitate surgery but may induce significant visual morbidity. Orbital tumors may induce proptosis, visual loss, relative afferent pupillary defect, disc edema and optic atrophy, but less than one-tenth of these tumors are confined to the optic nerve or its sheaths. No signs or symptoms are pathognomonic for tumors of the optic nerve. The tumors of the optic nerve may originate from the optic nerve itself (primary tumors) as a proliferation of cells normally present in the nerve (e.g., astrocytes and meningothelial cells). The optic nerve may also be invaded from tumors originating elsewhere (secondary tumors), invading the nerve from adjacent structures (e.g., choroidal melanoma and retinoblastoma) or from distant sites (e.g., lymphocytic infiltration and distant metastases). The purpose of this paper is to describe the various primary tumors of the optic nerve. In the first part of the review, the frequency, demographics and clinical presentation with systemic associations of the tumors are described, as well as the diagnostic features. In the second part, primarily tumor-specific histologic features are given. Finally, treatment modalities and prognosis are discussed.

Keywords: cancer; glioma; meningioma; optic nerve; orbit; pathology; tumor

Document Type: Research article

DOI: http://dx.doi.org/10.1586/eop.09.4

Affiliations: 1: Department of Neuroscience and Pharmacology, Eye Pathology Section, University of Copenhagen; and Department of Ophthalmology, Rigshospitalet, Copenhagen University Hospital, Copenhagen, Denmark., Email: jenslindegaard@gmail.com

Publication date: 2009-04-01

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