Optic nerve glioma

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Optic nerve glioma
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Magnetic resonance image of a large retrobulbar optic nerve tumor causing massive proptosis
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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. [1] [2]

Contents

Optic gliomas are usually pilocytic tumors, and can involve the optic nerve or optic chiasm. [3] Optic gliomas are usually associated with neurofibromatosis type 1 in 30% of people with the condition. [3]

Optic nerve gliomas have low mortality but extremely high prevalence of vision loss and eye-bulging exophthalmos) in children. [4] As of 2014, approximately 1000 cases had been reported. [4]

Diagnosis

Optic nerve gliomas are diagnosed using magnetic resonance imaging (MRI) and CT scans. [5] The tumor adopts a fusiform appearance, appearing wider in the middle and tapered at the ends. [5] Enlargement of the optic nerve along with a downward kink in the mid-orbit is usually observed. [5] While CT scans allow for optic nerve evaluation, MRI allows for intracranial evaluation to observe if the tumor has extended to other regions such as the optic chiasm and hypothalamus. [6]

Treatment

The main goal of treating optic gliomas is to preserve vision for as long as possible. [5] The tumor's slow and self-limiting growth indicates that it is not immediately problematic in most benign cases, with long-term studies showing that people with optic glioma may still have stable functional vision without intervention. [4] [6] [7] As a result, the first and preferred course of action is usually observation of optic glioma over time. [5] [6]

Once the first signs of visual deterioration and/or tumor progression are observed, interventional treatments will then commence. [6] These include radiation therapy, chemotherapy and surgical excision. While being the most effective therapy, radiation has shown damaging effects on the already compromised intellect as well as an increase in vascular issues and second tumor formation in children with neurofibromatosis 1. [4] However, fractionated stereotactic radiation therapy (FSRT) is gaining traction as the most preferred interventional treatment for optic nerve glioma due to its combination of the therapeutic efficiency of radiation therapy without the negative side effects. [6] Chemotherapy has also been shown to be a safer alternative to most radiation therapies and surgery for very young children (under the age of 3). [6] However, the optimal chemotherapeutic therapy has not been defined, with risks of different toxicities still observed in older children. [6] Surgery is considered the final choice of treatment, due to the high risk of blindness and damage to the affected eye. [4] [5] [6] It is considered in only certain scenarios, such as relieving a cosmetically unappealing bulging eye (exophthalmos), removing an enlarging and/or expanding tumor or a combination of both. [4] [6]

Prognosis

Optic gliomas alternate between periods of inactivity and growth, making their clinical presentation variable and clinical course unpredictable. [4] [5] Once the optic chiasm is involved, the prognosis for life and vision worsens. [6]

Related Research Articles

<span class="mw-page-title-main">Brain tumor</span> Neoplasm in the brain

A brain tumor occurs when abnormal cells form within the brain. There are two main types of tumors: malignant (cancerous) tumors and benign (non-cancerous) tumors. These can be further classified as primary tumors, which start within the brain, and secondary tumors, which most commonly have spread from tumors located outside the brain, known as brain metastasis tumors. All types of brain tumors may produce symptoms that vary depending on the size of the tumor and the part of the brain that is involved. Where symptoms exist, they may include headaches, seizures, problems with vision, vomiting and mental changes. Other symptoms may include difficulty walking, speaking, with sensations, or unconsciousness.

<span class="mw-page-title-main">Optic nerve</span> Second cranial nerve, which connects the eyes to the brain

In neuroanatomy, the optic nerve, also known as the second cranial nerve, cranial nerve II, or simply CN II, is a paired cranial nerve that transmits visual information from the retina to the brain. In humans, the optic nerve is derived from optic stalks during the seventh week of development and is composed of retinal ganglion cell axons and glial cells; it extends from the optic disc to the optic chiasma and continues as the optic tract to the lateral geniculate nucleus, pretectal nuclei, and superior colliculus.

<span class="mw-page-title-main">Papilledema</span> Eye disorder

Papilledema or papilloedema is optic disc swelling that is caused by increased intracranial pressure due to any cause. The swelling is usually bilateral and can occur over a period of hours to weeks. Unilateral presentation is extremely rare.

<span class="mw-page-title-main">Retinoblastoma</span> Medical condition

Retinoblastoma (Rb) is a rare form of cancer that rapidly develops from the immature cells of a retina, the light-detecting tissue of the eye. It is the most common primary malignant intraocular cancer in children, especially those under 3 years old.

<span class="mw-page-title-main">Glioma</span> Tumour of the glial cells of the brain or spine

A glioma is a type of primary tumor that starts in the glial cells of the brain or spinal cord. They are cancerous but some are extremely slow to develop. Gliomas comprise about 30 percent of all brain tumors and central nervous system tumours, and 80 percent of all malignant brain tumours.

Spinal tumors are neoplasms located in either the vertebral column or the spinal cord. There are three main types of spinal tumors classified based on their location: extradural and intradural. Extradural tumors are located outside the dura mater lining and are most commonly metastatic. Intradural tumors are located inside the dura mater lining and are further subdivided into intramedullary and extramedullary tumors. Intradural-intramedullary tumors are located within the dura and spinal cord parenchyma, while intradural-extramedullary tumors are located within the dura but outside the spinal cord parenchyma. The most common presenting symptom of spinal tumors is nocturnal back pain. Other common symptoms include muscle weakness, sensory loss, and difficulty walking. Loss of bowel and bladder control may occur during the later stages of the disease.

<span class="mw-page-title-main">Prolactinoma</span> Pituitary gland tumor

A prolactinoma is a tumor (adenoma) of the pituitary gland that produces the hormone prolactin. It is the most common type of functioning pituitary tumor. Symptoms of prolactinoma are due to abnormally high levels of prolactin in the blood (hyperprolactinemia), or due to pressure of the tumor on surrounding brain tissue and/or the optic nerves. Based on its size, a prolactinoma may be classified as a microprolactinoma or a macroprolactinoma.

<span class="mw-page-title-main">Glioblastoma</span> Aggressive type of brain cancer

Glioblastoma, previously known as glioblastoma multiforme (GBM), is the most aggressive and most common type of cancer that originates in the brain, and has a very poor prognosis for survival. Initial signs and symptoms of glioblastoma are nonspecific. They may include headaches, personality changes, nausea, and symptoms similar to those of a stroke. Symptoms often worsen rapidly and may progress to unconsciousness.

<span class="mw-page-title-main">Neurofibromatosis type I</span> Type of neurofibromatosis disease

Neurofibromatosis type I (NF-1), or von Recklinghausen syndrome, is a complex multi-system human disorder caused by the mutation of neurofibromin 1 (NF-1), a gene on chromosome 17 that is responsible for production of a protein (neurofibromin) which is needed for normal function in many human cell types. NF-1 causes tumors along the nervous system which can grow anywhere on the body. NF-1 is one of the most common genetic disorders and is not limited to any person's race or sex. NF-1 is an autosomal dominant disorder, which means that mutation or deletion of one copy of the NF-1 gene is sufficient for the development of NF-1, although presentation varies widely and is often different even between relatives affected by NF-1.

<span class="mw-page-title-main">Malignant peripheral nerve sheath tumor</span> Cancer of the connective tissue surrounding peripheral nerves

A malignant peripheral nerve sheath tumor (MPNST) is a form of cancer of the connective tissue surrounding peripheral nerves. Given its origin and behavior it is classified as a sarcoma. About half the cases are diagnosed in people with neurofibromatosis; the lifetime risk for an MPNST in patients with neurofibromatosis type 1 is 8–13%. MPNST with rhabdomyoblastomatous component are called malignant triton tumors.

<span class="mw-page-title-main">Pilocytic astrocytoma</span> Medical condition

Pilocytic astrocytoma is a brain tumor that occurs most commonly in children and young adults. They usually arise in the cerebellum, near the brainstem, in the hypothalamic region, or the optic chiasm, but they may occur in any area where astrocytes are present, including the cerebral hemispheres and the spinal cord. These tumors are usually slow growing and benign, corresponding to WHO malignancy grade 1.

<span class="mw-page-title-main">Optic nerve sheath meningioma</span>


Optic nerve sheath meningiomas (ONSM) are rare benign tumors of the optic nerve. 60–70% of cases occur in middle age females, and is more common in older adults. It is also seen in children, but this is rare. The tumors grow from cells that surround the optic nerve, and as the tumor grows, it compresses the optic nerve. This causes loss of vision in the affected eye. Rarely, it may affect both eyes at the same time.

<span class="mw-page-title-main">Sphenoid wing meningioma</span> Benign brain tumor

A sphenoid wing meningioma is a benign brain tumor near the sphenoid bone.

Pituitary apoplexy is bleeding into or impaired blood supply of the pituitary gland. This usually occurs in the presence of a tumor of the pituitary, although in 80% of cases this has not been diagnosed previously. The most common initial symptom is a sudden headache, often associated with a rapidly worsening visual field defect or double vision caused by compression of nerves surrounding the gland. This is often followed by acute symptoms caused by lack of secretion of essential hormones, predominantly adrenal insufficiency.

<span class="mw-page-title-main">Graves' ophthalmopathy</span> Medical condition

Graves' ophthalmopathy, also known as thyroid eye disease (TED), is an autoimmune inflammatory disorder of the orbit and periorbital tissues, characterized by upper eyelid retraction, lid lag, swelling, redness (erythema), conjunctivitis, and bulging eyes (exophthalmos). It occurs most commonly in individuals with Graves' disease, and less commonly in individuals with Hashimoto's thyroiditis, or in those who are euthyroid.

Optic neuropathy is damage to the optic nerve from any cause. The optic nerve is a bundle of millions of fibers in the retina that sends visual signals to the brain.

<span class="mw-page-title-main">Chiasmal syndrome</span> Set of signs and symptoms that are associated with lesions of the optic chiasm

Chiasmal syndrome is the set of signs and symptoms that are associated with lesions of the optic chiasm, manifesting as various impairments of the affected's visual field according to the location of the lesion along the optic nerve. Pituitary adenomas are the most common cause; however, chiasmal syndrome may be caused by cancer, or associated with other medical conditions such as multiple sclerosis and neurofibromatosis.

Within medical ophthalmology, Intraocular schwannoma, also termed uveal schwannoma, is a type of schwannoma found in the eye. These tumors are almost always benign in nature and while malignant forms have been documented in other areas of the body, this has not been reported in the uveal region. Composed of Schwann cells, these masses are generally slow growing and can be found in the peripheral nerve tract, often around the head and neck.

A central nervous system tumor is an abnormal growth of cells from the tissues of the brain or spinal cord. CNS tumor is a generic term encompassing over 120 distinct tumor types. Common symptoms of CNS tumors include vomiting, headache, changes in vision, nausea, and seizures. A CNS tumor can be detected and classified via neurological examination, medical imaging, such as x-ray imaging, magnetic resonance imaging (MRI) or computed tomography (CT), or after analysis of a biopsy.

<span class="mw-page-title-main">Visual pathway lesions</span> Visual maladies

The visual pathway consists of structures that carry visual information from the retina to the brain. Lesions in that pathway cause a variety of visual field defects. In the visual system of human eye, the visual information processed by retinal photoreceptor cells travel in the following way:
Retina→Optic nerve→Optic chiasma →Optic tract→Lateral geniculate body→Optic radiation→Primary visual cortex

References

  1. ↑ Huson, Susan Mary; Hughes, Richard Anthony Cranmer (1994). The neurofibromatoses: a pathogenetic and clinical overview. London: Chapman & Hall. 1.3.2:9. ISBN   0-412-38920-7.
  2. ↑ Skelley, Tao Le, Vikas Bhushan, Nathan William (2012-03-12). First aid for the USMLE step 2 CK (8th ed.). New York: McGraw-Hill Medical. ISBN   978-0-07-176137-6.{{cite book}}: CS1 maint: multiple names: authors list (link)
  3. 1 2 Goldman, Lee. Goldman's Cecil Medicine (24th ed.). Philadelphia: Elsevier Saunders. p. 1251. ISBN   1437727883.
  4. 1 2 3 4 5 6 7 Cameron, JD; Rodriguez, FJ; Rushing, E; Horkayne-Szakaly, I; Eberhart, C (2014). "An 80-year experience with optic nerve glioma cases at the Armed Forces Institute of Pathology: evolution from museum to molecular evaluation suggests possibe interventions in the cellular senescence and microglial pathways (an American Ophthalmological Society thesis)". Transactions of the American Ophthalmological Society. 112: 11–25. PMC   4234453 . PMID   25411512.
  5. 1 2 3 4 5 6 7 Shapey, J.; Danesh-Meyer, H.V.; Kaye, A.H. (December 2011). "Diagnosis and management of optic nerve glioma". Journal of Clinical Neuroscience. 18 (12): 1585–1591. doi:10.1016/j.jocn.2011.09.003. PMID   22071462. S2CID   29326564.
  6. 1 2 3 4 5 6 7 8 9 10 Nair, Akshay Gopinathan; Pathak, Rima S.; Iyer, Veena R.; Gandhi, Rashmin A. (16 April 2014). "Optic nerve glioma: an update". International Ophthalmology. 34 (4): 999–1005. doi:10.1007/s10792-014-9942-8. PMID   24736941. S2CID   13503634.
  7. ↑ Miller, NR (November 2004). "Primary tumours of the optic nerve and its sheath". Eye. 18 (11): 1026–37. doi: 10.1038/sj.eye.6701592 . PMID   15534587.
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