Malignant optic nerve glioma kennerdell case 53, ppt. The most common tumor of the optic nerve is optic nerve glioma, which represents approximately 66% of all primary optic nerve tumors. Approximately 40 cases of optic gbm in adults have been reported in the literature, and only five of. The visual system includes the optic nerve, the optic tract, the chiasm and the optic radiation. Clinically and histologically, these tumors were similar to the orbital optic nerve gliomas of childhood. Computed tomography and magnetic resonance imaging mri demonstrated enlargement of the optic chiasm and prechiasmic optic nerves. Optic nerve gliomas comprise about 1% of all intracranial tumours. Gliomas that affect the optic pathways are for the most part lowgrade neoplasms that often, but not always, have good prognoses. Radiation oncologyoptic pathway glioma wikibooks, open. Patient 1 was initially diagnosed with idiopathic retrobulbar optic neuritis. Optic nerve glioma with complete intraocular extension. Screening children with nf1 for optic pathway glioma yes eye. It is also unclear whether opg associated with neurofibromatosis nf is a distinct entity from nonnfopg. The involvement and distribution of the lesions are characteristic for optic pathway glioma opgs.
When tumor is confined to the optic nerves, ct imaging demonstrates well demarcated enlargement of the nerve, often with a tortuous or kinked appearance of the. Her visual acuity was moderately to severely reduced. This tumour type is named for its location on or near the optic nerve pathways between the eyes and the brain. Kennerdell, md, chair, department of ophthalmology, allegheny general hospital professor, ophthalmology, drexel university. Mainly, these lesions are inflammatory or vascular lesions that rarely necessitate surgery but may induce significant visual morbidity. We have seen 17 patients with optic nerve glioma in the past 12 years, and it has become clear that an appreciable proportion of them have had a progressive.
The present report of astrocytoma of the optic nerve head was unassociated with neurofibromatosis or tuberous. This system sends signals from the eye to the brain so a person can see images. The treatment of optic nerve glioma requires a multi. Relatively rare slow growing tumor within orbital segment of optic nerve usually ages 0 9 years with symptoms of minimal exophthalmos, optic nerve atrophy or papilledema.
Optic pathway glioma genetic and rare diseases information. Jan 27, 2015 gliomas that affect the optic pathways are for the most part lowgrade neoplasms that often, but not always, have good prognoses. Optic nerve glioma with complete intraocular extension sciencedirect. However, when the tumor extends to the optic chiasm or beyond, the prognosis worsens. Depending on how much pressure the tumor places on the optic nerve, the affected eye may bulge outward or twitch uncontrollably. Enter your email address to receive your free pdf download. Based on the imaging appearance, the tumour was categorised as.
French society of pediatric oncology, 2003 19901998 pmid 14673044 progressionfree survival in children with optic pathway tumors. Here, we present a case of highgrade glioma of the optic pathway with tramtracklike calcification of the optic nerve in a 42yearold male. This study aimed to determine if tortuosity and thickening are associated with the development of optic pathway glioma opg and subsequent vision loss. Optic pathway gliomas are relatively uncommon tumors, with a variable clinical course and usually seen in the setting of neurofibromatosis type i nf1. It is the purpose of this paper to report, in summary, the case histories of 34 patients with primary glioma of the optic nerves and chiasm and to evaluate the use of radiation therapy and surgery in their management. The differential diagnosis at this point was optic nerve meningioma versus optic nerve glioma. In seven cases, optic nerve glioma presented as an expanding orbital mass in previously asymptomatic adults. Visual outcome after proton beam irradiation depends on the height of the tumor and its location relative to the fovea and the optic nerve. Abstract the clinical presentation, diagnosis, response to therapy, and visual outcome of 18 children with optic nerve glioma are reported from the division of neurology, department of pediatrics, university of british columbia, vancouver, canada. Postcontrast mri showed marginal enhancement of the affected areas of the optic pathways. Approximately 90% of optic gliomas occur in children and most cases are relatively benign 18. We favor a treatment approach that is dependent on the location of tumor within. While it occurs in the pediatric and adult populations, clinical manifestations typically become apparent in childhood. Malignant optic gliomas are rare, but they rapidly become lethal visual pathway tumors.
There is a greater than 90% chance of survival after surgical resection when glioma has been limited to the optic nerve. The treatment of optic nerve gliomas has changed considerably over the past few years. A relationship between optic nerve glioma and nf1 is well established. We report a case of optic neuritis with enlargement of the optic nerve on mri. Thirtyone optic gliomas, 14 optic nerve meningiomas, and a single case of giant cell glioblastoma multiforme monstrocellular glioma of the optic chiasm were stained for agnors and counted in a masked fashion. B t2weighted mri with a hyperintense signal from the optic nerve glioma. Optic nerve gliomas were initially reported in less than 5% of nf1 cases, but this was before the advent of com puted tomography and magnetic resonance. Screening children with nf1 for optic pathway glioma yes. We present a case of sarcoidosis in a 14yearold girl who presented with a short history of visual disturbance.
Opgs are lowgrade glial neoplasms involving the visual pathway optic nerve, chiasm, tracts, and radiations and may also involve the hypothalamus. Patient 2 was found to have left optic nerve enhancement most consistent with an optic nerve glioma. Nine patients had a stable course with little change over a period of up to 8 years. Enlargement of the optic canal was present in 15 of the 16 patients examined, but this finding was unreliable as an indicator of the posterior extent of the tumour. However, orbital optic nerve gliomas rarely extend posteriorly after diagnosis. Optic nerve glioma and the of optic nerve tumours inthe. In adults, optic nerve gliomas do occur but are very rare and usually aggressive tumours 4. A glioma is a type of tumor originating in the brain or spinal cord that arises from the glial cells. Optic pathway gliomas in neurofibromatosis type 1 cynthia j. Dr praveen k tripathi optic pathway and hypothalamic glioma 23 may 2016 1 2. These cells form a tissue called glia that surrounds nerve.
Optimal treatment and management of optic pathway gliomas remains unclear and the decision hinges upon several factors including patient age, tumor location, and visual symptoms. Visual evoked potentials produced by was in eye vep. In the montage shown, sagittal contrastenhanced t1 and axial t2weighted magnetic resonance mr images demonstrate a pilocytic astrocytoma of the optic pathway. In the montage shown, sagittal contrastenhanced t1 and axial t2weighted magnetic resonance mr images demonstrate a. Intraocular extension of primary optic nerve gliomas has been described by many observers.
They include optic pathway glioma, medulloepithelioma. This tumor is strongly associated with neurofibromatosis 1 nf1 but can exist alone. The one patient explored via a lateral orbitotomy patient 85 had the optic nerve containing a large grade 1 astrocytoma excised. Click export csv or ris to download the entire page or use the checkboxes to select a subset of records to download. Wednesday, october 29, 2014 department of ophthalmology, jnmc 15 face photo of a 5yearold girl who developed noticeable proptosis od and found to harbor an optic nerve glioma. Mar 24, 2020 an individual who suffers from an optic nerve glioma is likely to experience some degree of vision impairment. The treatment of optic nerve glioma requires a multidisciplinary. The characteristic of ong is indolent growth with alternating periods of inactivity and growth with rare cases of spontaneous tumor regression. Optic nerve gliomas are rare tumors, accounting for 1. 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. Taken together, it is essential that future targeted therapies consider pediatric dosing and potential glioma resistance.
If symptomatic, signs include decreased visual acuity, proptosis, strabismus, and swelling of the optic disc. Unilateral malignant optic glioma following glioblastoma. A 21yearold man, who had gbm 7 years ago complained of a transient shadow in his vision and presented with normal visual acuity but. Evidencebased information on optic nerve glioma from hundreds of trustworthy sources for health and social care. Gliomas isolated to the optic nerve ong represent a subgroup of optic pathway gliomas, and their treatment remains controversial. Download pdf open epub full article content list abstract. Optic nerve glioma and the management british journal of. Optic nerve glioma and optic neuritis mimicking one. The tramtracklike calcification of the optic nerve, which classically occurs with meningioma, has not been reported so far in optic pathway gliomas. Orbital tumors may induce proptosis, visual loss, relative afferent pupillary defect, disc edema and optic atrophy, but less than onetenth of these tumors are confined to the optic nerve or its sheaths. Optic glioma definition of optic glioma by medical dictionary. She is suggested to do surgery by doctor, because after doing 10x chemo induction carbo vincris, although the glioma stable, but that fail to preserve. Biopsy should be avoided when possible due to the risk of visual impairment and high degree of uncertainty following histological analysis. Although atypical clinical progression led to further imaging studies, they did not provide the diagnosis because of inappropriate imaging protocols.
A 4yearold boy presented with a 1week history of left eye proptosis. An optic nerve glioma is a rare type of brain tumor found in the optic chiasm, where the optic nerves cross. Methods children with nf1 who underwent brain mri between 1992 and 2005, and had at. A more recent modification of the dodge classification has been proposed which glooma subdivides each stage 9. B t2 weighted mri with a hyperintense signal from the optic nerve glioma. An optic pathway tumor is one that is found along the visual system. Opg are often linked to neurofibromatosis type 1 nf1, see this term. T1weighted axial magnetic resonance images with gadolinium enhancement demonstrate an enhancing infiltrating mass extending from the right optic nerve to the chiasm and into the suprasellar space. Backgroundaims optic nerve tortuosity and nerve and sheath thickening are observed on mri in some patients with neurofibromatosis type 1 nf1. We report a 48yearold woman with a glioblastoma multiforme arising from the optic chiasm. Arachnoid hyperplasia is a meningeal response associated with gliomas of anterior optic pathway. Symptoms may include loss of vision, secondary strabismus, exophthalmos, and ocular paralysis. The extension indicates more agressive disease and poor clinical outcomes. Optic pathway glioma opg is a benign tumor that develop along the optic nerve chiasm, tracts, and radiations characterized by impairment or loss of vision and may be accompanied by diencephalic symptoms such as reduced growth and alteration in sleeping patterns.
The optic nerve glioma is isointense on this t 1weighted study. T1 mri with intravenous contrast and fat suppression is the imaging modality of choice for diagnosis. Jan 01, 2014 optic pathway glioma opg is a benign tumor that develop along the optic nerve chiasm, tracts, and radiations characterized by impairment or loss of vision and may be accompanied by diencephalic symptoms such as reduced growth and alteration in sleeping patterns. Chemotherapy and radiation therapy can now stabilize and in some cases improve the vision of patients with optic nerve gliomas.
These tumours may involve any part of the optic pathway and have the potential to spread along these. They can affect the optic nerve, optic chiasm, optic tracts and radiations and can either be sporadic or associated with neurofibromatosis type one. Since optic nerve gliomas are encountered infrequently, the effect of various forms of treatment is difficult to assess. Patients classically present in the fourth decade with a triad of vision loss, optic atrophy, and optociliary shunt vessels on fundoscopy. Optic nerve signal was also consistent with neuritis on the right. Optic nerve tumor an overview sciencedirect topics. Optic nerve glioma is typically a tumor of childhood. Apr 16, 2014 optic nerve glioma is the most common optic nerve tumour. A great deal has been written about the optimal approach to children with neurofibromatosis type 1 nf1. Pdf optic nerve glioma is the most common optic nerve tumour. In lowgrade optic nerve glioma, two divergent developments are observed. Primary tumours of the optic nerve and its sheath eye nature. We favor a treatment approach that is dependent on the location of. Despite the benign histology of optic pathway glioma opg lowgrade astrocytoma, its biological behavior is unpredictable, and it is unclear whether specific morphologic or anatomic patterns may be predictive of prognosis.
Download fulltext pdf download fulltext pdf download fulltext pdf. Treatment options for optic pathway gliomas springerlink. This lesion is amenable to surgery given the involvement of only one optic nerve with no extension to the optic chiasm. Interval increase of the optic nerve on magnetic resonance imaging prompted a biopsy of the optic nerve, which revealed a pilocytic astrocytoma. When tumor is confined to the optic nerves, ct imaging demonstrates welldemarcated enlargement of the nerve, often with a tortuous or kinked appearance of the. Hi friends, my daughter 11yo has optic pathway glioma. Malignant optic nerve glioma kennerdell case 53, ppt creator john s. Malignant optic gliomas are rare, and mostly present in. Posterior extension involving the optic chiasm and optic pathways is more common in sporadic cases compared to nf1 related glioma. Optic nerve glioma ong is the most common primary tumor of the optic nerve and is usually detected during the first. Children with nf1opgs involving the optic nerve extension into the chiasm and tracts permitted who.
Optic pathway gliomas in neurofibromatosis the authors 2017. Optic nerve glioma is the most common optic nerve tumour. Observation for clinical or radiographic stability or. Radiation therapy for optic nerve sheath meningioma. Tramtracklike calcification in adult optic pathway glioma. How should a childhood optic nerve glioma be worked up. Optic nerve sheath meningiomas onsms are usually benign neoplasms of the meninges surrounding the optic nerve. Optic nerve glioma an overview sciencedirect topics. Clinical features led to a misdiagnosis of optic neuritis in all cases.
Cooper, md, radiology medical group, san diego, california email protected. Peripheral vision loss is most common, but an advanced glioma can affect all aspects of sight. The main differential is that of optic nerve meningioma, however, the potential list is much longer including most causes of optic nerve enlargement. We evaluated the use of colloid silver impregnation of nucleolar organizer regionassociated proteins agnors in making this distinction. Optic gliomas are the most common tumor of the cns in nf1. Optic nerve glioma or optic glioma, a form of glioma which affects the optic nerve, is often one of the central nervous system manifestations of neurofibromatosis 1 optic gliomas are usually pilocytic tumors, and can involve the optic nerve or optic chiasm. Nucleolar organizer regions in optic gliomas brain. Primary tumours of the optic nerve and its sheath eye. Histologically the majority are pilocytic astrocytomas they are characterized by imaging by an enlarged optic nerve seen either on ct or mri.
An optic nerve glioma gleeohmuh is a type of brain tumor that forms in or around the optic nerve, which connects the eye to the brain. Definition the term optic pathway glioma is reserved for those tumors confined to the precortical visual pathway, sometimes with the involvement of the hypothalamus. Primary malignant gliomas of the optic nerve and chiasm are rare, and are usually rapidly fatal in adults. Neurofibromatosis type 1 and sporadic optic gliomas ncbi. Malignant optic and chiasmatic gliomas are extremely rare, and are classified pathologically as anaplastic astrocytoma or glioblastoma multiforme gbm. Management of optic nerve gliomas british journal of ophthalmology. Future research focused on defining the cellular and molecular factors that underlie optic glioma development and progression also has the potential to provide personalized risk assessment strategies for this pediatric population. The growth patterns of optic nerve gliomas are classified into intraneural and perineural forms. Optic nerve glioma is the most common type of tumor of this nerve. The absence of calcification can be used to differentiate optic nerve glioma from optic nerve sheath meningioma 6. To determine whether tumor size is associated with retinal nerve fiber layer rnfl thickness, a measure of axonal degeneration and an established biomarker of visual impairment in children with optic pathway gliomas opgs secondary to neurofibromatosis type 1 nf1. Children with optic glioma are usually screened for nf1 for this reason. Astrocytoma of the optic nerve head jama ophthalmology.
Neurosarcoidosis masquerading as glioma of the optic. Aug 22, 2015 wednesday, october 29, 2014 department of ophthalmology, jnmc 15 face photo of a 5yearold girl who developed noticeable proptosis od and found to harbor an optic nerve glioma. As such, generally, the term optic pathway glioma is favoured, recognising that there may be involvement of the hypothalamus. Adults with nf1 typically do not develop optic gliomas. The value of radiation therapy in the management of glioma. Optic nerve glioma ong is the most common primary tumor of the optic nerve and is usually detected during the. There has been no evidence of recurrence 12 years aftersurgery. Orbital optic nerve glioma in adult life jama ophthalmology. Optic gliomas are usually associated with neurofibromatosis type 1 in 30% of patients. This neuroradiological finding is most commonly seen in tumours of the optic nerve and led together with an inconclusive intraoperative frozen section to an incorrect tentative diagnosis of optic nerve glioma which fatally resulted in the resection of the optic nerve. We present the clinical course, treatment, and prognosis of a case of unilateral malignant optic glioma in a young man with a history of brain glioblastoma multiforme gbm.