Optic Pathway Tumor

What is an optic pathway tumor?

An optic pathway tumor is one that is found along the visual system. This system sends signals from the eye to the brain so a person can “see” images. The visual system includes the optic nerve, the optic tract, the chiasm and the optic radiation.

• Optic pathway tumors squeeze the visual system. This causes pressure, weakens the imaging signals sent to the brain, and damages vision.
• The extent of vision damage depends on two factors:
  • Location of the tumor
  • How early it is diagnosed—these tumors may grow rapidly or slowly
• The sooner the pressure is removed, the easier it is for vision to be restored—often completely.

How common are optic pathway tumors?

• More than 75% of these tumors occur during the first 10 years of life.
• Most are diagnosed in children age 5 or younger.
• Optic pathway tumors account for up to 5% of primary central nervous system tumors in children.
• Patients with a disease called neurofibromatosis 1 (NF-1) are at higher risk. Up to 20% of these children may also develop optic pathway tumors.

What are the symptoms of an optic pathway tumor?

Symptoms of an optic pathway tumor depend on the tumor’s location and the child’s age.

• Young children (especially those younger than 3) usually do not complain of vision loss until it is clear they need their eyes examined. By that time, the following symptoms may also be present:
  • Slower-than-normal growth
  • Tilting and bobbing of the head
  • Rapid eye movements (usually side to side)
  • Bulging eye
• Because the optic and endocrine systems are close to each other, optic pathway tumors may also affect areas controlled by the endocrine system, including the following:
  • Hormone production
  • Appetite
  • Sleep
  • Balance

How is an optic pathway tumor treated?

The treatment selected depends on the patient’s:

• Age,
• Extent of vision loss,
• Tumor location, and
• The presence of neurofibromatosis.

Chemotherapy, radiation, targeted therapy and surgery can all be used to treat optic pathway tumors. However, these tumors are complex. Many factors affect which treatment should be used. The St. Jude clinical team closely examines each case and creates a tailored approach for each patient depending on all the factors involved.

• Chemotherapy (“chemo”) — uses powerful medicines to kill cancer cells or stop them from growing (dividing) and making more cancer cells:
  • Chemo may be injected into the bloodstream, so that it can travel throughout the body.
  • Some chemo may be given by mouth.
  • Combination therapy uses more than one type of chemo at a time.
• Radiation therapy — uses high-energy X-rays or other types of radiation to kill cancer cells or stop them from growing:
  • External radiation uses machines outside the body to deliver the X-ray dose.
• Surgery — is used to remove tumor tissue. In general, optic pathway tumors can be treated without first testing a sample of the tumor to confirm what it is. These are the goals of surgery:
  • Complete removal of the tumor. Because of the location of these tumors, this is not possible for many children.
  • When the physical symptoms and radiology reports are not typical, surgery is needed to confirm exactly what these tumors are.
  • To preserve vision
  • Minimal or no eye damage

What are the survival rates for optic pathway tumors?

Most children with optic pathway tumors survive for a long time after diagnosis. Even so, these children may have serious visual, neurologic and other health problems. It is important that children with optic pathway tumors are treated in a center that focuses on quality of life as well as cures.

Why choose St. Jude for your child’s optic pathway tumor treatment?

• St. Jude has one of the largest pediatric brain tumor programs in the country.
• The St. Jude staff is dedicated to treating children who have serious illnesses. Here, you will find a hospital and staff that has only one focus—your child.
• St. Jude is committed to not only finding cures, but also helping children hold on to their quality of life. Children are not just small people. Their bodies and systems are unique, along with the disorders that affect them. For the best care, they need a specialized team. That is what you get from the St. Jude Brain Tumor Program.
• By working closely with the pediatric neurosurgeons at Le Bonheur Children’s Hospital in Memphis, the St. Jude Brain Tumor Program offers an expert team of doctors from many medical fields. The team includes: neuro-oncologists, who study and treat cancer in the brain and nervous system; neurosurgeons, who operate on the brain, skull, scalp and spinal cord; and radiation oncologists, who treat cancer with radiation. The team also includes neuropathologists, who can use certain tests and markers to provide the correct diagnosis. Without the right diagnosis there can be no proper treatment. Also, the team includes highly specialized neuro-radiologists who can provide a detailed report on the tumor status. All of these doctors have devoted their careers to finding cures and providing treatment for children with brain tumors. This diverse knowledge is vital to the complete care of children with brain tumors.
• Our brain tumor doctors also work closely with nursing, rehabilitation services, neuropathology, pharmacy, neuroradiology, neuropsychology, the school program, neurology, endocrinology, clinical nutrition, child life and social work, along with many others. Support staff members have been specially trained to care for children with brain tumors. We believe that providing holistic support for children and their families will ensure better quality of life.
• The nurse-to-patient ratio at St. Jude is unmatched— averaging 1:3 in hematology and oncology and 1:1 in the Intensive Care Unit.
• The St. Jude Brain Tumor Program has made great advances in treatments for brain tumor patients. Some of the world’s best neuroscientists, cancer biologists and doctors from the St. Jude Neurobiology and Brain Tumor Divisions combine their efforts to seek cures. In this interactive program, discoveries about the biology of both normal and harmful cell growth are rapidly tested in clinic studies. These studies include Phase I, II and III trials of the most common and deadly forms of childhood brain tumors.
• Being able to take a drug directly from the lab to your child is one of the major advantages of centers like St. Jude. Before treating a patient with any new drug, the St. Jude staff does many tests in the lab. The tests show if the drug is safe, if it works, and the best way to use it to help patients.
• St. Jude has created more clinical trials for cancer than any other children’s hospital in the United States.
• St. Jude provides access to different research studies that uses targeted therapy directed toward specific tumor markers that can be present in some cases of optic pathway tumors.
• Along with the treatment studies constantly being developing at St. Jude, the staff also treats patients on studies developed by the Children’s Oncology Group, the Pediatric Brain Tumor Consortium and the Pacific Pediatric Neuro-Oncology Consortium. Many St. Jude staff members play key roles in developing these studies, which are large tests carried out at centers across the United States.
• St. Jude practices patient family-centered care. Patient family-centered care is health care that focuses on the family as a child’s primary source of strength, support and well-being. At the heart of patient family-centered care is the belief that health care staff and the family are partners, working together to best meet the needs of the child. Excellence in health care happens when we work together and honor the expertise each of us brings to every health care encounter.
• St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.