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Definition

Retinoblastoma is a rare type of cancer found in the eye. In retinoblastoma, 1 or more tumors form in the retina. The retina is a layer of light-sensitive tissue that lines the back of the eye. It converts visual images into nerve impulses in the brain that allow us to see.

Retina of the Eye

Retina of the Eye

Copyright © Nucleus Medical Media, Inc.

As with most cancers, early diagnosis and treatment lead to better outcomes.

Causes

During early stages of fetal development, fast-growing, immature cells called retinoblasts form. Later, these cells become retinal cells. In retinoblastoma, some of these cells quickly grow out of control. If cells keep dividing uncontrollably when new cells are not needed, a mass of tissue forms, called a growth or tumor. The cancer may grow along the optic nerve and reach the brain, or it may travel to other parts of the body.

It is not clear exactly what causes these problems in the cells, but it is probably a combination of genetics and environment.

Risk Factors

Retinoblastoma is more common in children under 5 years old. Other factors that may increase your child's chance of retinoblastoma include:

  • Defect in retinoblastoma gene
  • Family members with retinoblastoma
Symptoms

Retinoblastoma usually forms only in 1 eye, but both eyes may have tumors. Symptoms are usually noticed by the parent or caregiver and may include, but are not limited to:

  • Eyes do not look normal, often described as a glazed look or a “cat’s eye.”
  • When a light is directed at the eye, the pupil looks white rather than red. This is often noticed on a photograph.
  • Eyes may appear to be crossed or looking in different directions.
  • Less common symptoms may include:
    • Eye may grow in size
    • Eye pain
    • Redness in the white part of the eye
    • Excessive tearing
    • Pupil may not respond to light
    • Iris (colored part of the eye) changes color
    • Double vision

Normal Anatomy of the Eye


Copyright © Nucleus Medical Media, Inc.

Diagnosis

The doctor will ask about symptoms and family medical history, and perform a physical exam. Many retinoblastomas are found during routine physical exams. If a tumor is suspected, your child will usually be referred to a specialist for a more complete eye exam. In children with a family history of the disease, eye exams often begin within 1-2 days of birth. Additional eye exams are scheduled at regular intervals thereafter.

Tests may include:

  • Dilated eye exam—To examine the inside of the eye with a lighted instrument.
  • Imaging test to evaluate the eye and surrounding tissue may include:

The physical exam combined with all of the test results, will help to determine the stage of cancer. Staging is used to guide your child's treatment plan. Like other cancers, retinoblastoma is staged from I-IV. Stage I is a very localized cancer, while stage IV indicates a spread to other parts of the body.

Treatment

Treatment depends on the stage of the cancer. Treatment aims to cure the cancer and preserve sight.

Therapies may be used alone or in combination, and may include:

Surgery

This involves surgical removal of the entire eye and as much of the optic nerve as possible. The optic nerve is the nerve leading from the eye to the brain that is responsible for vision. Surgery may be used for a large tumor in one eye.

Radiation Therapy

This treatment involves the use of radiation to kill cancer cells and shrink tumors. Radiation may be:

  • External radiation therapy—radiation directed at the tumor from a source outside the body
  • Internal radiation therapy—radioactive materials placed into the body near the cancer cells
Cryotherapy

Cryotherapy is the use of cold to freeze and destroy small tumors.

Thermotherapy

This treatment uses heat to kill cancer cells.

Photocoagulation

Lasers are used to destroy a small tumor.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells. This treatment may be given in many forms including pill, injection, and through a catheter. The drugs enter the bloodstream and travel through the body killing mostly cancer cells, but also some healthy cells.

Prevention

Genetic counseling and close monitoring and screening for people at risk for retinoblastoma can help prevent the disease or detect it early if it occurs.

Prevention and early detection techniques include:

  • Screening—Children born into families with a history of retinoblastoma should have regular eye exams to screen for development of the tumor. All children should have regular eye screening by their doctor.
  • Genetic counseling—This may help determine a person’s risk of developing retinoblastoma. If you have retinoblastoma or have a family history of the disorder, you can talk to a genetic counselor when deciding to have children.
  • Monitoring for recurrence—People who have been treated for retinoblastoma require regular medical exams to assess the success of treatment and check for recurrence or bilateral disease.
  • Monitoring for other cancers—Children with retinoblastoma are at increased risk for an associated brain tumor and other cancers in the body.

RESOURCES:

American Cancer Society
http://www.cancer.org

National Cancer Institute
http://www.cancer.gov

CANADIAN RESOURCES:

BC Cancer Agency
http://www.bccancer.bc.ca

Cancer Care Ontario
http://www.cancercare.on.ca

References:

Retinoblastoma. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated September 13, 2013. Accessed November 8, 2013.

Clinical Oncology. 2nd ed. Churchill Livingstone, Inc; 2000.

Nelson Textbook of Pediatrics. 16th ed. WB Saunders Co; 2000.

Ophthalmology. Mosby International Ltd; 1999.

Principles and Practice of Pediatric Oncology. 3rd ed. Lippincott-Raven Publishers; 1997.



Last reviewed October 2014 by Eric L. Berman, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.

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