Optic nerve glioma associated with neurofibromatosis type 1. Optic nerve gliomas adopt one of the 2 growth patterns. There is a greater than 90% chance of survival after surgical resection when glioma has been limited to the optic nerve. This tumor is strongly associated with neurofibromatosis 1 nf1 but can exist alone. Other than optic nerve and visual pathway gliomas, children with neurofibromatosis are at increased likelihood of developing a variety of different brain tumors.
An optic nerve glioma is a tumor that appears on the structure that carries visual input from the eye to the brain. As the tumour grows it affects the nerves, causing problems with vision. Optic nerve glioma and the management of optic nerve. The period of greatest risk for the development of symptomatic optic gliomas in nf1 is during the first 6 years of life. The fusiform enlargement of the right sided intra orbital part of optic nerve result in moderate rightside proptosis. An optic glioma may also grow along with a hypothalamic glioma. This child developed visual difficulties and was discovered to have a glioma nerve tumor in the optic nerve. Optic nerve glioma is a slow growing tumour developing within the optic nerve. Links to pubmed are also available for selected references. Age 2 years, 2 months the size of the optic glioma was 5mm. Our patient underwent a frontal craniotomy approach for optic nerve glioma on december 29, 1982. While it occurs in the pediatric and adult populations, clinical manifestations typically become apparent in childhood. Unilateral adult malignant glioma of the optic nerve is exceptional. Clinically and histologically, these tumors were similar to the orbital optic nerve gliomas of childhood.
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. This is a single case and should be interpreted with caution. 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. 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. Patients who have malignant gliomas of the optic nerve have rapidly progressive, painful visual loss accompanied by.
B, axial t2weighted mri scan noncontrastenhanced of the orbits shows an enlarged, hyperintense, globular glioma of the right optic. Optic pathway gliomas opg are considered relatively benign pediatric tumors. They can affect the optic nerve, optic chiasm, optic tracts and radiations and can either be sporadic or associated with neurofibromatosis type one. In seven cases, optic nerve glioma presented as an expanding orbital mass in previously asymptomatic adults. Primary tumours of the optic nerve and its sheath eye. Another name for this tumour is a pilocytic astrocytoma. Optic nerve optic pathway glioma symptoms, causes and. Treatment of glioma of the optic nerve depends on the spread of the tumor in the rear direction. About 33 percent of all brain tumors are gliomas, which originate in the glial cells that. Glioma is a common type of tumor originating in the brain. Age 2 years, 9 months the size of the optic glioma was 6mm. The treatment of optic nerve gliomas has changed considerably over the past few years. Optic nerve gliomas are rare tumors, accounting for 1.
This xray shows the skull of a child with neurofibromatosis nf1. The term optic glioma refers to glial tumors that involve the anterior visual pathwayoptic nerves, optic chiasm, and optic tracts. 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. Understanding inherited genetic risk of adult glioma a. Optic nerve glioma optic gliomas are the most common tumor of the cns in nf1. Most gliomas, especially those found in young children, are benign and easily treated.
There were only 2 pediatric cases, both classified as b cell lymphoma. We suggest a method of management of primary optic nerve tumours, both meningiomas and gliomas, in young patients. If symptomatic, signs include decreased visual acuity, proptosis, strabismus, and swelling of the optic disc. More commonly, there is intraneural glial proliferation as neoplastic growth occurs within individual nerve fascicles. Spontaneous regression has been documented with optic nerve gliomas. The optic nerve was crosssectioned and proven to be a complete resection, yet retinal circulation. Some tumors in adults, however, quickly turn cancerous, grow aggressively, and spread to other parts of the brain and body.
Optic glioma, one of the most significant complications of nf1 in childhood, developed with an approximate prevalence of 15% range, 1. Symptoms may include loss of vision, secondary strabismus, exophthalmos, and ocular paralysis. Optic glioma is a brain tumor that occurs in and around the optic nerves. These tumors are composed primarily of pilocytic astrocytes. The other pattern is characterized by perineural arachnoidal gliomatosis pag with invasion of the leptomeninges and relative sparing of the optic nerve. Arachnoid hyperplasia is a meningeal response associated with gliomas of anterior optic pathway.
Computerized tomography may show concentric enlargement of the optic canal in patients with onh. Diagnosis was made by optic nerve biopsy in 12 cases and at autopsy in 4 more. The treatment of optic nerve gliomas is controversial. The articles prior to january 20 are part of the back file collection and are not available with a current paid subscription. For full access to this pdf, sign in to an existing account, or purchase an annual subscription. Her visual acuity was moderately to severely reduced. The ct scan confirmed presence of optic nerve glioma ong. Gliomas can affect all ages, but they are most often seen in adults. Division of neuroepidemiology, department of neurological surgery.
The tramtracklike calcification of the optic nerve, which classically occurs with meningioma, has not been reported so far in. Optic glioma definition of optic glioma by medical. 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. Additionally, when the bulk of a tumor is located at the chiasm, the differential should include pituitary region masses. Gliomas of the optic pathways that occur in adults are rare tumours. 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. Unilateral adult malignant optic nerve glioma springerlink. Both forms of optic nerve glioma can result in partial or total vision. The treatment of optic nerve glioma requires a multi. Click to download and get the free sample pdf file of the report.
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. The final diagnosis was only confirmed by optic nerve biopsy. Typically, each type of tumor is named after the kinds of cells it affects. Any surgical maneuver within the subarachnoid space puts the optic nerve at risk. Melanocytomas are typically a benign meaning they can grow, but rarely transform into a malignancy. Gliomas isolated to the optic nerve ong represent a subgroup of optic pathway gliomas, and their treatment remains controversial. Pdf role of dti optic pathway in glioma find, read and cite all the research you need on researchgate.
An optic nerve glioma is a rare type of brain tumor found in the optic chiasm, where the optic nerves cross. The absence of calcification can be used to differentiate optic nerve glioma from optic nerve sheath meningioma 6. Optic nerve glioma an overview sciencedirect topics. Optic nerve glioma 2074 computed tomography ct scan of the brain and orbit was done figure 3. However, when the tumor extends to the optic chiasm or beyond, the prognosis worsens.
Optic nerve glioma american academy of ophthalmology. Glioma of the optic nerve symptoms and treatment of. This tumor type is common and frequent in children with a genetic condition, known as neurofibromatosis. Obviously, tumors in both categories affect the visual system, but the two types differ in pace and progres sion. 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.
Optic nerve glioma and the of optic nerve tumours inthe. Optic pathway gliomas in neurofibromatosis the authors 2017. Visual evoked potentials produced by was in eye vep. As the tumor progresses, it presses on the optic nerve, causing a childs vision to worsen. Most optic nerve gliomas are considered lowgrade and dont grow as quickly as other types of brain tumors. 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. Purpose to describe the clinical, neuroimaging, and pathologic features of primary tumours of the optic nerve and its sheath. Optic gliomas are usually pilocytic tumors, and can involve the optic nerve or optic chiasm. Pdf file of the complete article 408k, or click on a page image below to browse page by page. Observation is indicated in the absence of signs of growth, good vision and lack of cosmetic defects.
Right side demonstrates proliferating glial cells of central intraneural portion of tumor hematoxylineosin, original magnification x120. Optic neuropathy in the context of leukemia or lymphoma. Gliomas are slightly more likely to occur in men than in women, and more common in caucasians than in african americans. Full text get a printable copy pdf file of the complete article 1. Blindness can occur, but only in about 5 percent of cases. Optic nerve glioma is the most common optic nerve tumour. Chemotherapy and radiation therapy can now stabilize and in some cases improve the vision of patients with optic nerve gliomas. Patients who get this tumour commonly have an under lying medical condition called neurofibromatosis type 1.
The optic nerve is enlarged and kinked and demonstrates mild hypodense cystic change centrally. A, axial contrastenhanced orbital ct scan shows a right optic nerve glioma. To characterize the clinical course of adult patients with opg. Optic nerve glioma is the most common type of tumor of this nerve. Optic gliomas are the most common tumor of the cns in nf1.
847 1577 1505 517 166 364 650 331 1359 229 633 1460 1375 212 610 1294 1206 743 721 763 439 230 1386 319 196 512 218 587 1379 203