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Retinoblastoma

Definition

Retinoblastoma is a rare type of cancer found in the eye. In retinoblastoma, one 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.
As with most cancers, early diagnosis and treatment lead to better outcomes.
Retina of the Eye
Retina of the Eye
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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 called a tumor grows. 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:

Symptoms

Retinoblastoma usually forms only in one eye, but both eyes may have tumors. Symptoms are usually noticed by the parent or caregiver and may include, but are not limited to:
Normal Anatomy of the Eye
IMAGE
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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:
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 directed at the tumor from a source outside the body
  • Internal—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:

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

Canadian Cancer Society http://www.cancer.ca

References

Retinoblastoma. EBSCO DynaMed website. Available at: http://www.ebscohost.com/dynamed. Updated September 13, 2013. Accessed November 6, 2015.

Retinoblastoma. Merck Manual Professional Version website. Available at: http://www.merckmanuals.com/professional/pediatrics/pediatric-cancers/retinoblastoma. Updated August 2015. Accessed November 6, 2015.

What is retinoblastoma? American Academy of Ophthalmology Eye Smart website. Available at: http://www.geteyesmart.org/eyesmart/diseases/retinoblastoma.cfm. Accessed November 6, 2015.

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