It is a rare disease with the most common signs being Leukocoria (white pupil) and misaligned eyes (strabismus). In other cases, children may develop neovascular glaucoma, which can cause enlargement of the eye (buphthalmos).
According to the American Cancer Society, about 300 children are diagnosed with retinoblastoma each year in the United States. Retinoblastoma is more common in infants and very young children than in older children. The average age of diagnosis is eighteen months and rarely occurs in children older than six.
Studies reveal that retinoblastoma occurs about equally in boys and girls and in different races and ethnicities. It also occurs equally in the right or left eye.
TYPES OF DIAGNOSIS: Retinoblastoma is categorized into intraocular or extraocular disease. Intraocular retinoblastoma is localized to the eye and may be confined to the retina, but does not extend beyond the eye into the tissues around the eye or to other parts of the body.
Extraocular disease, on the other hand, extends beyond the eye. It may be confined to the tissues around the eye, or it may spread to the central nervous system or most commonly to the bone marrow or lymph nodes.
Children with neovascular glaucoma and enlargement of the eye are at greater risk for extraocular spread of their retinoblastoma. (SOURCE: Eye Cancer Network, National Cancer Institute)
TREATMENT: Treatment of retinoblastoma often requires a team of doctors made up of ophthalmic, radiation and pediatric oncologists. Treatment has evolved from the removal of the eye, to eye-sparing radiotherapy, and more recently to chemotherapy-based therapy.
Chemotherapy shrinks the retinoblastoma in order to treat them with laser therapy, freezing therapy (cryotherapy), and local "plaque" radiation. These techniques are thought to be safer than external beam radiation.
External radiation therapy effectively treats retinoblastoma, but it can cause side effects because the radiation often reaches nearby tissues as well.
The American Cancer Society noted that doctors are continually improving instruments used for cryotherapy, laser therapy, and other local treatments. The goal is to more precisely kill tumor cells while sparing other parts of the eye.
According to the Eye Cancer Network, more than 90 percent of children can be cured of retinoblastoma by early detection and treatment of the affected eye. Unfortunately, some children can have both eyes affected.
FAMILY HISTORY: Retinoblastoma was the first cancer to be directly associated with a genetic abnormality. Retinoblastoma can occur sporadically or inherited. If a genetic mutation is found there is a 45-50 percent chance that the parents will have another child with retinoblastoma. If there is no family history and no mutation is found, the risk of having a second child with retinoblastoma is 2-5 percent. (SOURCE www.kidsgrowth.com)
FOR MORE INFORMATION, PLEASE CONTACT:
Judy Martin
Associate Director of Media Relations
Washington University School of Medicine
(314) 286- 0105
martinju@wustl.edu
