[Pineal tumor involving simultaneously the pineal body and optic tract].
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A 58-year-old woman with a 35-year history of left proptosis underwent neuroimaging that revealed a large cystic lesion. Surgery revealed an optic nerve sheath meningioma associated with cyst formation. The cyst was part of the tumor, a phenomenon that is well described in intracranial meningiomas but not in optic nerve sheath meningiomas.
PURPOSE OF REVIEW: Recent trends in the management of optic nerve sheath meningiomas have shifted towards fractionated conformal external beam radiotherapy. The purpose of this paper is to review the current literature on this subject and review the clinical characteristics and natural history of optic nerve sheath meningiomas. RECENT FINDINGS: Articles published within the last 2 years have reported the efficacy of using fractionated radiation therapy alone. A total of 62 patients in three studies have been reported; 40.3% retained stable visual acuity, 41.2% percent had improvement in their vision, and 18.4% percent had a decrease in visual acuity. Median follow-up time ranged from 22 to 51 months in these studies. SUMMARY: Optic nerve sheath meningiomas may be safely managed with fractionated external beam radiotherapy in select patients with possible improvement or stabilization of visual acuity. This represents an enormous improvement in a disease with typically poor visual prognosis.
PURPOSE: To describe a role for optic nerve decompression as adjuvant surgical therapy in the management of optic nerve sheath meningioma in patients with severe, progressive visual loss and optic disc edema before or after radiation therapy. METHODS: Interventional case report. RESULTS: Two patients with unilateral optic nerve sheath meningioma had progressive visual loss (20/200 and no light perception) and disc edema. The first had previously undergone fractionated stereotactic radiotherapy and the second subsequently was treated with fractionated stereotactic radiotherapy after decompression surgery. After excision of a dural window and biopsy of the tumor from the nerve sheath, visual acuity improved to 20/25 and 20/200, respectively, both coinciding with resolution of disc edema. CONCLUSIONS: In patients with optic nerve sheath meningioma with severe disc edema and rapid vision loss, surgery may serve an important but restricted, adjuvant role to radiation therapy under special conditions.
PURPOSE: To study the natural history and growth of optic nerve sheath meningiomas and evaluate their management outcome. DESIGN: Clinicopathologic retrospective noncomparative case series. METHODS: A retrospective study of 88 patients who were treated between 1976 and 1999 at the University of British Columbia and the University of Amsterdam. Clinical reports, imaging studies, and histopathologic findings were reviewed. RESULTS: The mean age at onset of symptoms was 40.3 years, and most were seen in middle-aged females. Patients typically presented with visual loss, frequently associated with optic atrophy or papilledema and occasionally optociliary shunt vessels. On imaging, the optic nerve demonstrated segmental or diffuse thickening of the sheath or globular growth. Calcification was seen in 31% of cases and was associated with slower tumor growth. Tumors with posterior components in the orbit had more frequent intracranial involvement. Intracranial extension was more frequent and had a greater growth rate in younger patients. Irregular margins in the orbit implied local invasion. A presenting visual acuity better than 20/50 correlated with longer preservation of vision. Patients who underwent radiotherapy showed improvement in their visual acuity, and tumor growth was halted. Optic sheath decompression did not preserve vision. En bloc tumor excision was associated with no detectable recurrence in contrast to debulked tumors that recurred. CONCLUSIONS: Meningiomas show characteristic indolent growth. Management therefore should be conservative in most cases. Radiotherapy is indicated in patients with progressive visual deterioration. Surgery, when indicated, should be an en bloc excision.
The authors describe the case of bilateral optic nerve sheath meningioma /ONSM/ in 14 years old girl. When being hospitalized at the Neurology Clinic with paresis n.VII, the patient was examined by ophthalmologist. The diagnosis was made on the basis of clinical and characteristic changes revealed by USG, CT and MRI. The very first ophthalmologic examination confirmed a visual acuity 5/5 OD and hand movements OS, associated with papilloedema OD and edematous optic atrophy OS. The use of USG, CT and MRI revealed bilateral tubular thickening of the optic nerves (8mm), with calcification. After the period of one year, clinical progression was observed. The intracranial tumor extended. It was shown in diagnostic imaging. The patient was operated on by neurosurgeons with histopathology verification. In the later stage she underwent radiotherapy. The case has been described because of its diagnostic and therapeutic difficulties.
We describe six cases of cannalicular optic nerve meningioma in which the diagnosis was missed for more than 1 year after the onset of symptoms. Clinical features led to a misdiagnosis of optic neuritis in all cases. Although atypical clinical progression led to further imaging studies, they did not provide the diagnosis because of inappropriate imaging protocols. Diagnosis was eventually made on the basis of high-spatial-resolution contrast-enhanced MR findings. Radiologists should have a high suspicion for the diagnosis of optic canal meningioma in patients with unexplained visual loss, particularly when visual loss is progressive. Investigation in these cases should include high-spatial-resolution MR imaging of the orbit before and after contrast medium administration, and fat suppression should be used in combination with contrast enhancement whenever possible.
OBJECTIVE: To describe the natural history of patients with primary optic nerve sheath meningioma (ONSM) who were cared for without intervention. METHODS: A retrospective review of the medical records of 42 patients identified in the database of an academic neuro-ophthalmology unit who had been diagnosed with a unilateral ONSM. Twenty-five had been referred after treatment had been implemented, and 1 was blind at referral. The remaining 16 patients who were followed up with observation only are the focus of this study. RESULTS: The study subjects were followed up for a mean of 6.2 years (range, 2-18 years). The mean follow-up from the time of the first symptom was 10.2 years (range, 3-28 years). No patient died or incurred neurological deficits other than vision loss. At diagnosis, 12 of 16 had a visual acuity of 20/100 or better; 11 had a visual acuity of 20/30 or better. At follow-up, 8 of 16 had a visual acuity of 20/100 or better; 6 had a visual acuity of 20/30 or better. Three patients had slight improvement. Visual fields remained stable in 4 patients and improved in the 3 patients whose visual acuity also improved. CONCLUSIONS: Some patients with ONSM have a stable course for many years, and a few may even show slight improvement. The routine application of radiation therapy may unnecessarily expose some patients to complications and should be reserved for those patients whose visual function declines under observation.
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OBJECTIVE AND IMPORTANCE: The clinical and radiographic presentations of optic nerve gliomas and optic neuritis are for the most part distinct and their diagnoses straightforward. We present two cases illustrating the occasional difficulty one can encounter in distinguishing neoplastic from inflammatory optic neuropathies. CLINICAL PRESENTATION: Patient 1 is a 17-year-old girl who presented with acute onset of pain and rapidly progressive visual loss in the right eye. Patient 2 is a 38-year-old man who presented with painless progressive visual loss in the left eye. INTERVENTION: Patient 1 was initially diagnosed with idiopathic retrobulbar optic neuritis. Interval increase of the optic nerve on magnetic resonance imaging prompted a biopsy of the optic nerve, which revealed a pilocytic astrocytoma. Patient 2 was found to have left optic nerve enhancement most consistent with an optic nerve glioma. Before a biopsy, the patient spontaneously improved without treatment, indicating an inflammatory process. CONCLUSION: Differentiating between optic nerve neoplasm and inflammation may be difficult. On occasion, the classic clinical finding of pain with eye movement and the radiographic finding of enlargement and enhancement of the optic nerve may be misleading. Open biopsy of the optic nerve is indicated only after a completely negative metabolic, infectious, and inflammatory workup; interval increase of the optic nerve on magnetic resonance imaging; and failure of the patient to recover vision.
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