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A childhood optic pathway tumor occurs along the nerve that sends messages from the eye to the brain (the optic nerve). These tumors may grow rapidly or slowly, depending on the grade of the tumor. More than 75 percent of these tumors occur during the first decade of life. Most are diagnosed in children 5 or younger.
Optic pathway tumors constitute up to 5 percent of primary central nervous system tumors in children.
An association with neurofibromatosis is present in 50-70 percent of patients with isolated optic nerve tumors and in 16-20 percent of patients with chiasmal or deeper optic tract tumors.
Signs and symptoms depend on the specific location of the tumor and the age of the patient. Young children usually don’t complain of the slow and progressive visual loss that is characteristic of these tumors. Children younger than 3 are usually taken to the doctor because of obvious developmental delays. Infants display head tilt, head bobbing, and nystamus (oscillation of the eyeballs.) Growth and endocrine disturbances can also be symptoms of optic pathway tumors.
Greatest risk is loss of vision in one or both eyes, but overall, survival is near 90 percent.
Surgery, chemotherapy, and radiation are all treatment options for optic pathway tumors. The precise combination of therapies depends on the age of the patient, extent of visual loss, and the location of the tumor.
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