Retinoblastoma
Definition
Retinoblastoma is a malignant tumor (cancer) of the retina (part of the eye) that generally affects children under the age of 6. It is most commonly diagnosed in children aged 1 - 2 years.
Alternative Names
Tumor - retina; Cancer - retina
Causes
Retinoblastoma occurs when a cell of the growing retina develops a mutation, causing it to grow out of control and become cancerous.
Sometimes this mutation develops in a child whose family has never had eye cancer, but other times the mutation is present in several family members. If the mutation runs in the family, there is a 50% chance that an affected person's children will also have the mutation. They will therefor have a high risk of developing retinoblastoma themselves.
One or both eyes may be affected. A visible whiteness in the pupil may be present. Blindness can occur in the affected eye. The eyes may appear crossed. The tumor can spread to the eye socket through the optic nerve. It may also spread to the brain, lungs and bones. This is a rare tumor, except in families that carry the RB gene mutation.
Symptoms
Exams and Tests
Treatment
Treatment options depend upon the size and location of the tumor. Small tumors may be treated by laser surgery. Radiation and chemotherapy may be needed if the tumor has spread beyond the eye.
The eye may need to be removed if the tumor does not respond to other treatments. It is important to seek treatment from a physician with experience treating this rare type of tumor.
Outlook (Prognosis)
If the cancer has not spread beyond the eye, almost all patients can be cured. A cure, however, may require aggressive treatment and even removal of the eye in order to be successful. If the cancer has spread beyond the eye, the likelihood of a cure is lower and depends on how the tumor has spread.
Possible Complications
Spread of the tumor to the brain or other locations can occur.
When to Contact a Medical Professional
Call your health care provider if signs or symptoms of retinoblastoma are present, especially if your child's eyes look abnormal or appear abnormal in photographs.
Prevention
Genetic counseling can help families understand the risk of retinoblastoma. It is especially important when more than one family member has had the disease, or if the retinoblastoma occurs in both eyes.
References
Chintagumpala M, Chevez-Barrios P, Paysse EA, Plon SE, Hurwitz R. Retinoblastoma: review of current management. Oncologist. Oct 2007;12(10):1237-46.
Melamud A. Retinoblastoma. Am Fam Physician. Mar 15, 2006;73(6):1039-44.
Review Date:
6/10/2008
Reviewed By:
James R. Mason, MD, Oncologist, Director, Blood and Marrow Transplantation Program and Stem Cell Processing Lab, Scripps Clinic, Torrey Pines, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.
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