This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Citing articles on Web of Science (74)  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

Jack Stern, MD, PhD; Frederick A. Jakobiec, MD; Edgar M. Housepian, MD

Arch Ophthalmol. 1980;98(3):505-511.

Abstract
• A retrospective clinicopathologic study of 34 patients with optic nerve glioma disclosed two important architectural tumor forms: circumferential-perineural pattern featuring tumor eruption and proliferation in the subarachnoid space correlating with the presence of neurofibromatosis, and an expansile-intraneural pattern correlating with the absence of neurofibromatosis. The 18 patients with neurofibromatosis had a mean age of 4.9 years at presentation and tended to be younger than the 16 patients without neurofibromatosis (mean age, 12 years). Instead of "arachnoidal hyperplasia," electron microscopic studies in the circumferential-perineural pattern showed tumor astrocytes admixed with reactive meningothelial cells, fibroblasts, and collagen in distended subarachnoid space. Florid invasion of the leptomeninges (arachnoidal gliomatosis) exhibited by many optic nerve gliomas in neurofibromatosis suggests that they are true neoplasms rather than hamartomas, but their frequent location in the distal anterior visual pathway often confers a good clinical prognosis.

Author Affiliations
From the Neurological Institute of New York (Drs Stern and Housepian), and the Algernon B. Reese Laboratory of Ophthalmic Pathology of the Edward S. Harkness Institute (Dr Jakobiec), Columbia-Presbyterian Medical Center, New York. Dr Jakobiec is now with the Manhattan Eye, Ear, and Throat Hospital and the New York Hospital-Cornell Medical Center, New York.

Footnotes
Accepted for publication June 11, 1979.

Reprint requests to Department of Pathology, Manhattan Eye, Ear, and Throat Hospital, 210 E 64th St, New York, NY 10021 (Dr Jakobiec).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    Twitter     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

Perineural gliomatosis associated with isolated optic nerve gliomas
Yeung et al.
Br J Ophthalmol 2009;93:839-841.
FULL TEXT  

From the Archives of the AFIP: Pediatric Orbit Tumors and Tumorlike Lesions: Neuroepithelial Lesions of the Ocular Globe and Optic Nerve
Chung et al.
RadioGraphics 2007;27:1159-1186.
ABSTRACT | FULL TEXT  

Screening and Diagnosis of Optic Pathway Gliomas in Children with Neurofibromatosis Type 1 by Using Sweep Visual Evoked Potentials
Chang et al.
IOVS 2007;48:2895-2902.
ABSTRACT | FULL TEXT  

From the Archives of the AFIP: Pilocytic Astrocytoma: Radiologic-Pathologic Correlation
Koeller and Rushing
RadioGraphics 2004;24:1693-1708.
ABSTRACT | FULL TEXT  

Prognostic factors of CNS tumours in Neurofibromatosis 1 (NF1): A retrospective study of 104 patients
Guillamo et al.
Brain 2002;126:152-160.
ABSTRACT | FULL TEXT  

MRI and clinical differences between optic pathway tumours in children with and without neurofibromatosis
Chateil et al.
Br. J. Radiol. 2001;74:24-31.
ABSTRACT | FULL TEXT  

Orbital Optic Glioma in Neurofibromatosis: Magnetic Resonance Diagnosis of Perineural Arachnoidal Gliomatosis
Seiff et al.
Arch Ophthalmol 1987;105:1689-1692.
ABSTRACT