Patients classically present in the fourth decade with a triad of vision loss, optic atrophy, and optociliary shunt vessels on fundoscopy. Optic nerve sheath meningioma jama neurology jama network. Recent reports have emphasised the efficacy of fractionated stereotactic or conformal radiotherapy, and some clinicians favour this approach instead of surgery or observation. Optic nerve sheath meningioma is a benign not malignant tumor which involves the covering of the optic nerve. Typically, it affects middleaged women with an average age of 41 years.
Clinical features led to a misdiagnosis of optic neuritis in all cases. A sagittal t1 weighted gadolinium enhanced image showed a normal sized left optic nerve, concentrically surrounded by a homogeneously enhancing mass extending from the globe to the optic foramen figure a. The use of three dimensional 3d ultrasonography 3dus for optic nerve measurements has been described in normal eyes utilising coronal cscans. Optic nerve sheath meningiomas originate from arachnoid cap cells within the dural sheath surrounding the intraorbital or less commonly, the intracanalicular optic nerve. Oct 24, 2018 optic nerve sheath meningiomas are rare benign neoplasms of the meninges surrounding the optic nerve.
Primary onsm represents a neoplasia of meningothelial cap cells of. Optic nerve sheath meningiomas represent approximately 2% of all orbital tumors, as reported in large orbital tumor series by wright et al. Lippincott journals subscribers, use your username or email along with your password to log in. Optic nerve sheath meningiomas onsms grow slowly and, if untreated, patients may have stable visual function for up to several years. Optic nerve sheath meningiomas classically are seen in middleaged or elderly females and present as slowly progressing axial proptosis and gradual loss of. Diagnosis and treatment of orbital optic nerve sheath. Meningiomas along with gliomas and pituitary tumor are the most common tumors inside the skull. Treatment of an onsm may lead to vision loss radiation retinopathy or optic neuropathy. Surgery revealed an optic nerve sheath meningioma associated with cyst formation. Optic nerve sheath meningioma masquerading as optic neuritis. Notice that the tumor is relatively bright radiodense.
Case report optic nerve sheath meningioma masquerading as. My present meningioma is behind my right eye next to my optic nerve. Purpose to describe the clinical, neuroimaging, and pathologic features of primary tumours of the optic nerve and its sheath. A classification system for onsm according to the tumor location has been established by schick et al. The presentation of optic nerve sheath meningioma onsms largely depends on whether they arise from the orbit, within the optic canal, or intracranially. We report the case of a 39yearold woman presenting with bilateral meningocele uncovered during a routine examination for headache complaints.
Diagnosis and treatment of orbital optic nerve sheath meningioma. Pdf optic neuritis is a common presentation of demyelinating disorders such as multiple sclerosis. Primary tumours of the optic nerve and its sheath eye. The best treatment of optic nerve sheath meningiomas remains controversial. Meningioma, optic nerve, optic nerve sheath, radiotherapy introduction optic nerve sheath meningiomas onsms account for onethird of primary optic nerve tumours, are the second most common optic nerve tumours after gliomas, and are the most common tumours of the optic nerve sheath. The authors are to be complimented on their removal of a subdural optic nerve sheath meningioma. Visual outcome indeed seemed better in radiotherapy management than. Historically, treatments included resection or observation, both leaving patients with poor functional outcomes. A patient with bilateral idiopathic optic nerve sheath inflammation, described by margo et al, 8 did not experience a beneficial response to corticosteroids. Diosgenin inhibits the proliferation, migration and. When these tumors are left untreated, however, progressive visual impairment ensues, which also leads to blindness. Optic nerve sheath meningioma onsm originates from meningothelial cap cells in the arachnoid membrane that surrounds the optic nerve 15. On coronal views, the tumor demonstrates a doughnut shape, with the dense nerve sheath tumor encir cling the optic nerve figs.
Any optic nerve meningioma patients whove had surgery. Optic nerve sheath meningiomas onsms represent the most common tumors of the optic nerve sheath and account for onethird of primary optic nerve tumors. Histopathological examination confirmed the clinical diagnosis of an. This longstanding untreated optic nerve sheath meningioma extended intracranially, widened the optic canal and became continuous with other durally based meningiomas. Various surgical procedures, depending on the extent of the tumor, have been practiced in patients with primary optic nerve sheath meningioma. To explain the concepts that radiation oncologists should understand to manage patients with optic nerve sheath meningioma onsm. A fullthickness biopsy specimen of the more severely involved optic nerve revealed chronic inflammation of the optic nerve sheath with vasculitic changes and necrobiotic granulomas. Most of these tumours are benign but malignant gliomas of the optic nerve occur as do other tumours, including gangliogliomas, medulloepitheliomas, and vascular tumours, such as haemangioblastomas and haemangiopericytomas. To accomplish this goal we first describe our institutions experience with the treatment of onsm with radiation therapy and then use this data set as a framework for explaining the role of radiation therapy in the treatment of this uncommon tumor. The management of primary optic nerve sheath meningioma onsm is controversial. Optic nerve sheath meningioma onsm usually arises from the intraorbital part of the optic nerve sheath and accounts for approximately 2% of all orbital tumor. Therefore, observation is recommended for a patient with onsm and relatively preserved visual acuity, color vision, pupils, and visual fields. If untreated, they can involve the chiasm and extend into the contralateral optic nerve. Retrospective analysis of fractionated intensitymodulated.
An xray film shows the linear density seen on the right side of the film crossing the inferior orbital rim which corresponds to the optic nerve sheath meningioma seen above. Current diagnosis and management of optic nerve sheath. Two cases secondary to a small meningioma in the orbital apex and one secondary to an intraorbital optic nerve sheath meningioma are. Surgery often results in postoperative blindness in the affected eye and thus has been abandoned as a treatment option for most patients.
Pdf optic nerve sheath meningioma masquerading as optic. Case report optic nerve sheath meningioma masquerading as optic neuritis r. May 18, 2016 the term optic nerve sheath meningioma onsm does not indicate a definite site of origin. Optic nerve sheath meningioma dr daniel j bell and assoc prof frank gaillard et al. However, we take issue with their comments relative to a prior article. Optic nerve sheath meningiomas onsm are rare benign tumors of the optic nerve. Primary orbital meningiomas, originating in the optic nerve sheath, represent 12% of all meningiomas, and are the second most common optic nerve tumor after gliomas. Optic nerve sheath meningiomas and optic nerve gliomas can be difficult to differentiate by computed tomography ct.
Optic nerve sheath meningiomas onsms are rare tumors of the anterior visual pathway and constitute approximately 2% of all orbital tumors and 12% of all meningiomas. The most common tumour of the optic nerve is the optic nerve glioma. These are slow growing, leading to problems when they interfere with normal function. It is a remnant from my original atypical meningioma that was removed on 4292015, by neurosurgeon matthew hummel in austin, tx. Stereotactic irradiation of biopsy proved optic nerve sheath. Optic nerve sheath meningioma radiology reference article.
In each case, an abnormal optic nerve sheath was identified by ct. Optic nerve sheath meningioma journal of neurology. Although atypical clinical progression led to further imaging studies, they did not provide the diagnosis because of inappropriate imaging. The natural history of onsms typically shows progressive, painless visual decline. Mri applied to primary and secondary meningioma of the study of the orbital cavities was performed. Untreated onsms almost always lead to progressive visual decline, color blindness, and finally complete loss of vision. Behbehani 2,3 division of neurology, department of medicine, amiri hospital, p. New concepts in the management of optic nerve sheath meningiomas. Total recovery of optic nerve sheath meningioma bmj case. He didnt say it but i think he left the remnant because it was too close to my optic nerve. Involvement of the contralateral side in the absence of nf2 is infrequent, although dutton reported that 38% of his patients had this extent of disease if the meningioma had spread to involve the nerve sheath within the optic canal. Optic nerve sheath meningiomas nonsurgical treatment. A softtissue mass arose from one side of the sheath.
Poptic nerve sheath meningiomas onsms are rare tumors of the anterior visual pathway and constitute approxi. Note the almost calcific density of the left optic nerve. Primary optic nerve sheath meningioma onsm is a rare benign tumour of anterior visual pathways whose delayed diagnosis can lead to progressive visual loss and blindness in advanced cases. Extirpation, sometimes including removal of the optic nerve from chiasm to globe, have occasionally been suggested. To accomplish this goal we first describe our institutions experience with the treatment of onsm with radiation therapy and then use this data set as a framework for explaining the role of radiation therapy in the treatment of this. The shunt vessels are seen in only a third of optic nerve meningiomas. It was pressing on my optic nerve and i was told i would lose the sight in my left eye very soon and the right eye eventually. Primary onsms account for approximately one third of primary optic nerve tumors and 5% to 10% of orbital tumors 1, 2. New concepts in the management of optic nerve sheath. Computed tomography ct shows that the eye is pushed forward by this optic nerve sheath meningioma. These tumors typically appear as masses within the optic nerve, isointense.
Jun 01, 2016 computed tomography ct shows that the eye is pushed forward by this optic nerve sheath meningioma. Optic nerve sheath meningiomas onsms are usually benign neoplasms of the meninges surrounding the optic nerve. Intracanicular optic nerve involvement may also occur in association with a meningioma of the anterior clinoid or tuberculum sellae. Nerve sheath meningioma optic nerve sheath meningioma onsm is a term applied to primary and secondary meningiomas of the optic nerve. The optic nerve sheath may secondarily become affected by an intracranial meningioma on or near the planum sphenoidale that spreads anteriorly. The cyst was part of the tumor, a phenomenon that is well described in intracranial meningiomas but not in optic nerve sheath meningiomas. Radiation therapy for optic nerve sheath meningioma. To the editor we read with interest the report on possible optic nerve meningioma by ebers et al archives 1980. Discussion a differential imaging diagnosis should be made with. A 58yearold woman with a 35year history of left proptosis underwent neuroimaging that revealed a large cystic lesion. Cysts of the optic nerve three cases associated with.
The extent of involvement of the optic nerve can be difficult to determine even with mri. The tumors grow from cells that surround the optic nerve, and as the tumor grows, it compresses the optic nerve. The triad of visual loss, optic atrophy, and optociliary shunt veins is characteristic of an optic nerve sheath meningioma. Optic nerve meningiomas appear on mri as a fusiform enlargement of the optic nervesheath complex. Onsm is a relatively rare tumor and represents 12% of all intracranial meningiomas 4,69. Primary optic nerve sheath meningiomas clinical gate. Detection and treatment of optic nerve sheath meningioma. 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. The optic nerve was atrophied and there was significant proptosis. Stereotactic irradiation of biopsy proved optic nerve. The tumor may arise from either the intraorbital or intracanalicular portions of the optic nerve sheath. Imaging of the brain can determine if the meningioma extends into the brain. Three patients with optic nerve sheath meningiomas were studied with a ctt 8800 scanner with 1.
Notwithstanding, the classic triad of onsm is visual loss, optic atrophy, and optociliary shunt vessels. In the images above, note that computed tomography of this optic nerve sheath meningioma. Optic nerve sheath meningiomas onsms represent 1% to 2% of all meningiomas, 1. Isolated optic nerve sheath meningocele is a rare affection defined as the cystic enlargement of the optic nerve sheath filled with cerebrospinal fluid. Although atypical clinical progression led to further imaging studies, they did not provide the diagnosis because of inappropriate imaging protocols. However, we take issue with their comments relative to. New concepts in the diagnosis and management of optic nerve sheath meningioma. Optic nerve sheath meningioma onsm is a term applied to primary and secondary meningioma of the optic nerve. Optic nerve sheath meningioma an overview sciencedirect. In the current research work, the anticancer potential of diosgenin was evaluated in optic nerve sheath meningioma cells along with evaluating its effects on cancer cell invasion, migration, apoptotic cell death, autophagy and cell cycle progression. Optic nerve sheath meningiomas arise from the meningothelial cap cells of arachnoid villi and can develop anywhere along the course of the optic nerve from the globe to the prechiasmal intracisternal optic nerve.
Optic nerve meningiomas are benign tumors arising from the arachnoid cap cells of the optic nerve sheath and represent 20% of all orbital meningiomas, the majority of which are direct extensions from intracranial meningiomas. The term optic nerve sheath meningioma onsm does not indicate a definite site of origin. Primary onsms arise from the cap cells of the arachnoid surrounding the intraorbital or, less frequently, the intracanalicular optic nerve. Optic nerve sheath meningiomas represent only 1% to 2% of meningiomas 2 but pose considerable clinical challenges owing to their intimate association with the optic nerve and its vasculature. Optic nerve sheath meningioma new york eye cancer center. Optic nerve sheath meningiomas onsm are rare, benign neoplasms originating from the meningothelial cells of the meninges surrounding the optic nerve. Computed tomography of the optic nerve sheath meningioma. While the mortality rate is practically zero, these tumors can blind or disfigure patients. Visual outcome indeed seemed better in radiotherapy management than neurosurgery with less late. According to a recent metaanalysis and subsequently reported large biinstitutional series, primary onsms accounts for approximately one third of primary optic nerve tumors and 5% to 10% of orbital tumors. Fundoscopy and thy optic nerve sheath meningioma onsm is a term roid hormones still revealed no abnormal findings.
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