Childhood cancers

Retinoblastoma

Retinoblastoma is a rare cancer of the retina of the eye that is usually found in children, though it can occur at any age. The cancer may involve one or both eyes, and it is usually confined to the eye.

As with all cancers, retinoblastoma is best treated in the early stages. A goal of treatment is to destroy the cancer while preserving as much vision as possible. About 300 children are diagnosed with retinoblastoma in the U.S. each year. The incidence is about equal between boys and girls.

Survival rates are very good, with more than 93 percent of children surviving five years after diagnosis.

Risk factors
A gene has been identified as causing some cases of retinoblastoma. Children who inherit the gene have a 90 percent risk of developing the disease. Hereditary cases usually occur in very young children and often involve both eyes. As a precaution, other siblings of a child with retinoblastoma should also have regular eye examinations. In addition, parents may wish to consider genetic consultation to better understand the nature of genetic diseases. Children with retinoblastoma may also have an increased risk of developing osteosarcoma.

Cases that occur in older children usually affect only one eye and are most likely not hereditary.

Symptoms
Sometimes, retinoblastoma can be seen by looking into the child's eye. It is usually detected during an examination by an ophthalmologist—a doctor who specializes in diseases of the eyes. The pupil (dark center) of the eye may have a white glow or spots, the iris may be a different color in each eye, or the eye may be red and sore with poor vision.

Questions to ask your child's doctor

Diagnosis
Retinoblastoma is usually diagnosed in a hospital setting after placing the child under general anesthesia and looking at the eye with specialized instruments.

Treatment
Before cancer is treated, it has to be staged. Staging involves finding out where the cancer is located, the extent of the cancer and whether it has spread. Early stage cancers (Stage 1 and 2 that have not spread) are the easiest to treat.

Retinoblastoma is generally a slow-growing tumor and is best treated when the disease is localized (confined to the eye). The goal is to destroy the tumor using radiation therapy while preserving normal vision. If both eyes are involved, great effort is made to preserve vision in at least one eye. Sometimes, the tumor is so large the eye must be removed.

It is important for the child to be evaluated by several specialists as early as possible so that treatment may be coordinated effectively from the beginning. Depending on the stage, these specialists may include an ophthalmologist, radiation oncologist, radiologist, medical oncologist and a pathologist.

In advanced stages, the cancer can spread to other parts of the body and more extensive treatment, including chemotherapy and radiation therapy, may be required.

Clinical trials, protocols and research

Learn more
• YNHH Health Library: Retinoblastoma

Last revised: Jan. 8, 2008 (dh)

Home page URL: http://www.ynhh.org

All information is intended for your general knowledge and is not a substitute for medical advice or treatment for specific medical conditions. You should seek prompt medical care for any specific health issues and consult your physician before starting a new fitness regimen.