Purpose to describe the clinical, neuroimaging, and pathologic features of primary tumours of the optic nerve and its sheath. Gliomas can affect all ages, but they are most often seen in adults. The optic nerve is enlarged and kinked and demonstrates mild hypodense cystic change centrally. Optic nerve glioma an overview sciencedirect topics. As the tumour grows it affects the nerves, causing problems with vision. Both forms of optic nerve glioma can result in partial or total vision.
Optic gliomas are usually pilocytic tumors, and can involve the optic nerve or optic chiasm. Additionally, when the bulk of a tumor is located at the chiasm, the differential should include pituitary region masses. Blindness can occur, but only in about 5 percent of cases. The treatment of optic nerve glioma requires a multi. Optic nerve glioma and the management of optic nerve. Diagnosis was made by optic nerve biopsy in 12 cases and at autopsy in 4 more. Our patient underwent a frontal craniotomy approach for optic nerve glioma on december 29, 1982. An optic pathway glioma also called an optic nerve glioma is a slowgrowing brain tumor that arises in or around the optic nerve, which connects the eye to the brain. Age 2 years, 2 months the size of the optic glioma was 5mm.
Optic pathway gliomas are lowgrade neoplasms that affect the precortical visual pathway of children and adolescents. Treatment of glioma of the optic nerve depends on the spread of the tumor in the rear direction. Most optic nerve gliomas are considered lowgrade and dont grow as quickly as other types of brain tumors. This tumor type is common and frequent in children with a genetic condition, known as neurofibromatosis. 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 is controversial. Age 2 years, 9 months the size of the optic glioma was 6mm. Patients who get this tumour commonly have an under lying medical condition called neurofibromatosis type 1. The tramtracklike calcification of the optic nerve, which classically occurs with meningioma, has not been reported so far in. Optic gliomas are the most common tumor of the cns in nf1. Optic nerve glioma is a slow growing tumour developing within the optic nerve. Optic nerve glioma is the most common type of tumor of this nerve. If symptomatic, signs include decreased visual acuity, proptosis, strabismus, and swelling of the optic disc. While it occurs in the pediatric and adult populations, clinical manifestations typically become apparent in childhood.
B, axial t2weighted mri scan noncontrastenhanced of the orbits shows an enlarged, hyperintense, globular glioma of the right optic. Typically, each type of tumor is named after the kinds of cells it affects. However, when the tumor extends to the optic chiasm or beyond, the prognosis worsens. Visual evoked potentials produced by was in eye vep. Notably, 1 biopsy report noted that the optic nerve sheath was not infiltrated, 8 demonstrating that biopsy of the nerve itself is required to confidently rule out lymphomatous infiltration. The treatment of optic nerve gliomas has changed considerably over the past few years. Gliomas isolated to the optic nerve ong represent a subgroup of optic pathway gliomas, and their treatment remains controversial. There were only 2 pediatric cases, both classified as b cell lymphoma.
An optic nerve glioma is a tumor that appears on the structure that carries visual input from the eye to the brain. Optic nerve glioma 2074 computed tomography ct scan of the brain and orbit was done figure 3. Melanocytomas are typically a benign meaning they can grow, but rarely transform into a malignancy. Pdf file of the complete article 408k, or click on a page image below to browse page by page.
Optic nerve glioma associated with neurofibromatosis type 1. Optic glioma definition of optic glioma by medical. Right side demonstrates proliferating glial cells of central intraneural portion of tumor hematoxylineosin, original magnification x120. Imaging that exposes these optice nerve gliomas may also reveal the high signal intensity lesions in the basal ganglia, thalamus, brainstem, cerebellum, or subcortical white matter consistent with cns hamartomas.
Other than optic nerve and visual pathway gliomas, children with neurofibromatosis are at increased likelihood of developing a variety of different brain tumors. The final diagnosis was only confirmed by optic nerve biopsy. The presenting symptoms and signs, management, and prognosis may vary, depending on whether the tumor is confined to one optic nerve or involves the optic chiasm. Her visual acuity was moderately to severely reduced. Chemotherapy and radiation therapy can now stabilize and in some cases improve the vision of patients with optic nerve gliomas. An optic nerve glioma is a rare type of brain tumor found in the optic chiasm, where the optic nerves cross. Ashleys oncologist believes its prudent to not start chemo too early, but if your daughters optic glioma is growing, it may be time to start chemo. This is not a specific sign, however, as it can be seen in many conditions where the optic nerve is enlarged, most commonly optic nerve glioma. Pdf role of dti optic pathway in glioma find, read and cite all the research you need on researchgate. An optic nerve melanocytoma is a tumor made up of melanocytes and melanin. The absence of calcification can be used to differentiate optic nerve glioma from optic nerve sheath meningioma 6.
Patients who have malignant gliomas of the optic nerve have rapidly progressive, painful visual loss accompanied by. Gliomas are slightly more likely to occur in men than in women, and more common in caucasians than in african americans. Optic nerve glioma and the of optic nerve tumours inthe. Arachnoid hyperplasia is a meningeal response associated with gliomas of anterior optic pathway. Optic glioma, one of the most significant complications of nf1 in childhood, developed with an approximate prevalence of 15% range, 1. The term optic glioma refers to glial tumors that involve the anterior visual pathwayoptic nerves, optic chiasm, and optic tracts. About 33 percent of all brain tumors are gliomas, which originate in the glial cells that.
Optic nerve glioma optic gliomas are the most common tumor of the cns in nf1. Click to download and get the free sample pdf file of the report. Optic nerve glioma american academy of ophthalmology. The optic nerve was crosssectioned and proven to be a complete resection, yet retinal circulation. The fusiform enlargement of the right sided intra orbital part of optic nerve result in moderate rightside proptosis. Optic pathway gliomas opg are considered relatively benign pediatric tumors. Optic nerve optic pathway glioma opg an optic nerve glioma also known as an optic pathway glioma opg is a slowgrowing brain tumour that forms around the nerves that carry information from the eye to the brain. The other pattern is characterized by perineural arachnoidal gliomatosis pag with invasion of the leptomeninges and relative sparing of the optic nerve. We suggest a method of management of primary optic nerve tumours, both meningiomas and gliomas, in young patients. Glioma is a common type of tumor originating in the brain. Glioma of the optic nerve symptoms and treatment of. The period of greatest risk for the development of symptomatic optic gliomas in nf1 is during the first 6 years of life. Computerized tomography may show concentric enlargement of the optic canal in patients with onh.
Full text get a printable copy pdf file of the complete article 1. Any surgical maneuver within the subarachnoid space puts the optic nerve at risk. 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. An optic glioma may also grow along with a hypothalamic glioma. Spontaneous regression has been documented with optic nerve gliomas. This is a single case and should be interpreted with caution. Links to pubmed are also available for selected references.
This child developed visual difficulties and was discovered to have a glioma nerve tumor in the optic nerve. Obviously, tumors in both categories affect the visual system, but the two types differ in pace and progres sion. Unilateral adult malignant optic nerve glioma springerlink. Another name for this tumour is a pilocytic astrocytoma.
Observation is indicated in the absence of signs of growth, good vision and lack of cosmetic defects. Optic nerve gliomas are rare tumors, accounting for 1. Optic glioma is a brain tumor that occurs in and around the optic nerves. Optic pathway gliomas in neurofibromatosis the authors 2017.
Optic neuropathy in the context of leukemia or lymphoma. The articles prior to january 20 are part of the back file collection and are not available with a current paid subscription. Gliomas of the optic pathways that occur in adults are rare tumours. Most gliomas, especially those found in young children, are benign and easily treated.
To characterize the clinical course of adult patients with opg. As the tumor progresses, it presses on the optic nerve, causing a childs vision to worsen. Symptoms may include loss of vision, secondary strabismus, exophthalmos, and ocular paralysis. These tumors are composed primarily of pilocytic astrocytes. For full access to this pdf, sign in to an existing account, or purchase an annual subscription. Optic nerve sheath decompression has been reported to be beneficial in treating optic nerve glioma, although in our patient there was no appreciable egress of csf when the optic nerve sheath was opened. Division of neuroepidemiology, department of neurological surgery. Optic nerve optic pathway glioma symptoms, causes and. This xray shows the skull of a child with neurofibromatosis nf1.
The most common are gliomas in different portions of the brain, including the brain stem, and the cerebral cortex the area of the brain in charge of language and information processing. A, axial contrastenhanced orbital ct scan shows a right optic nerve glioma. They can affect the optic nerve, optic chiasm, optic tracts and radiations and can either be sporadic or associated with neurofibromatosis type one. Unilateral adult malignant glioma of the optic nerve is exceptional. This tumor is strongly associated with neurofibromatosis 1 nf1 but can exist alone. Primary tumours of the optic nerve and its sheath eye. Optic nerve glioma is the most common optic nerve tumour. In seven cases, optic nerve glioma presented as an expanding orbital mass in previously asymptomatic adults.