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. If not treated, the tumors will continue growing. The cancer may grow along the optic nerve and reach the brain or it may travel to other parts of the body.
Cancer occurs when cells in the body (in this case retina cells) divide without control or order. Normally, cells divide in a regulated manner. If cells keep dividing uncontrollably when new cells are not needed, a mass of tissue forms, called a growth or tumor. Tumors can invade nearby tissue and spread to other parts of the body.
Retina of the Eye
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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. Some cases of the tumor are inherited; others are not. Retinoblastoma usually forms only in one eye, but both eyes may have tumors.
A risk factor is something that increases your chance of getting a disease or condition. Risk factors for retinoblastoma include:
Symptoms are usually noticed by the parent or caregiver and may include, but are not limited to:
Normal Anatomy of the Eye
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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, the 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 a day or two of birth. Additional eye exams are scheduled at regular intervals thereafter.
Once retinoblastoma is found, staging tests are performed to find out if the cancer has spread, and, if so, to what extent. Treatment depends on the stage of the cancer. The cancer may be localized to the eyes or it may have spread to tissues around the eye or to other parts of the body.
Tests may include, but are not limited to:
General anesthesia may be given to keep the child still during close examination and testing.
Your child will likely be referred to a specialist for treatment. Without treatment, the cancer cells will continue to grow.
Treatment aims to cure the cancer and preserve sight. Options vary, depending on whether the disease is limited to the eye or has spread, and how large and where in the eye the tumor is located. Therapies may be used alone or in combination.
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.
This treatment involves the use of radiation to kill cancer cells and shrink tumors. Radiation may be:
Used on small tumors, cryotherapy is the use of cold to freeze and destroy cancer cells.
This treatment uses heat to kill cancer cells.
Lasers are used to destroy a small tumor.
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.
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.
Early diagnosis and treatment improve the chance of successful treatment. Prevention and early detection techniques include:
American Cancer Society
National Cancer Institute
BC Cancer Agency
Cancer Care Ontario
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 [Under Medical Review] 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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