Disease Information

Brain Tumor: Optic Pathway Tumor

Definition

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.

Incidence

Optic pathway tumors constitute up to 5 percent of primary central nervous system tumors in children.

Influencing Factors

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.

Clinical Features and Symptoms

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.

Survival Rates

Greatest risk is loss of vision in one or both eyes, but overall, survival is near 90 percent.

Treatment Strategies

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.

Current Research

• New radiation techniques that minimize damage to healthy tissue that surrounds brain tumor tissue are under investigation.
  
• Stem cell transplantation as a part of treatment continues to be under study.
  
• Scientists continue to study chromosomal abnormalities, genes, and proteins that may have a role in the development and metastasis (spread to other parts of the central nervous system) of pediatric brain tumors.
  
• Clinical trials are underway to help develop chemotherapy drugs that are more effective against this tumor.

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