Seeing is Deceiving: Optic Neuritis Parading as Glioma

Journal of Neurology, Neurosurgery & Psychiatry, 1986

Thirty five adults and two children with clinically isolated optic neuritis were examined by magnetic resonance imaging (MRI) to determine the presence of disseminated lesions within the brain at presentation and to compare these findings with the results of evoked potential studies. Of the adult patients, 61% showed lesions on the scans whereas the evoked potentials suggested the presence of lesions outside the visual system in 30%. MRI is a sensitive method for the demonstration of clinically unsuspected lesions in patients with uncomplicated optic neuritis.

Neuro-Ophthalmology, 2017

A 49-year-old previously healthy woman presented with acute painless visual loss in the right eye, a right relative afferent pupillary defect, and a normal fundus examination. She was diagnosed with retrobulbar "optic neuritis" and given a course of intravenous steroids. Despite treatment, however, she continued to lose vision and serial visual field testing confirmed a junctional scotoma in the fellow eye. Cranial magnetic resonance imaging (MRI) showed a mass at the junction between the right optic nerve and the anterior aspect of the chiasm and a right frontal lesion that proved to be multicentric glioblastoma multiforme. Clinicians should be aware of the possibility of aggressive neoplasm in the differential diagnosis of retrobulbar optic neuritis.

Surgical Neurology International, 2014

Background: Arachnoid proliferation, although rare, is known to occur in association with optic gliomas. However, chondroid and chordoid metaplasia has not been reported previously. Case Description: A 27-year-old male presented with progressive, painless loss of vision in right eye, associated with vomiting and headache for one and a half months. Computed tomography (CT) scan revealed a contrast enhancing mass arising from planum sphenoidale. Perioperative findings showed the tumor adherent to the right optic nerve and attached to basal dura and falx. A clinical impression of an intradural, optic nerve sheath meningioma was made. Histopathological examination revealed a glial tumor with adjacent areas displaying marked fibroblastic and arachnoid cell proliferation with chondroid as well as chordoid differentiation along with myxoid change and dense collagenisation. Reticulin stain, immunochemistry with glial fibrillary acid protein (GFAP), epithelial membrane antigen (EMA), and S-100 helped to arrive at the final diagnosis of optic glioma displaying exuberant arachnoid proliferation with cartilaginous metaplasia. Conclusion: We report a case of optic nerve glioma displaying extensive arachnoid proliferation, chordoid, and cartilaginous metaplasia, which mimicked chondrosarcoma or chordoid meningioma, posing a diagnostic dilemma. A clinical feedback, simple reticulin stain, and GFAP staining is of immense value in such cases to arrive at the correct diagnosis.

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),

Purpose: To present a rare presentation of optic nerve glioma (ONG) with total intraocular extension. Methods: A 44-year-old man with a history of loss of vision since childhood and recent development of progressive disfiguring proptosis was referred. Results: The vision in the affected eye was no light perception, and a proptosis of about 2e3 mm was present. Computed tomography and Magnetic Resonance Imaging (MRI) of brain/orbit showed a fusiform enlargement of the left optic nerve and total filling of vitreous cavity by a mass with high signal intensity in T2-weighted MRI. There was no extension into the intracranial cavity. The patient also had no signs of neurofibromatosis. Histopathology confirmed the diagnosis of ONG of the left orbit with extension into the globe and filling vitreous cavity. Conclusion: ONG may extend to the vitreous cavity with no simultaneous intracranial involvement.

Interdisciplinary Neurosurgery, 2020

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International Ophthalmology, 2014

Optic nerve glioma is the most common optic nerve tumour. However, it has an unpredictable natural history. The treatment of optic nerve gliomas has changed considerably over the past few years. Chemotherapy and radiation therapy can now stabilize and in some cases improve the vision of patients with optic nerve gliomas. The treatment of optic nerve glioma requires a multidisciplinary approach where all treatment options may have to be implemented in a highly individualized manner. The aim of this review article is to present current diagnostic and treatment protocols for optic nerve glioma.

Journal of Clinical Neuroscience, 2011

Graefe's Archive for Clinical and Experimental Ophthalmology, 2015

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.

Canadian Journal of Ophthalmology / Journal Canadien d'Ophtalmologie