Retinoblastoma

Retinoblastoma at a Glance

• Retinoblastoma is a disease in which malignant (cancer) cells form in the tissues of the retina.
• Retinoblastoma is a relatively uncommon tumor of childhood
• Although retinoblastoma may occur at any age, it most often occurs in younger children, usually before age of 5 years.

About retinoblastoma

The retina is the nervous tissue that lines the inside of the back of the eye. The retina senses light and sends images to the brain by way of the optic nerve.
Retinoblastoma is a relatively uncommon tumor of childhood that arises in the retina and accounts for about 3% of the cancers occurring in children younger than 15 years. The estimated annual incidence in the United States is approximately 10 to 14 per million children aged 0 to 4 years.

Although retinoblastoma may occur at any age, it usually occurs in children younger than 5 years of age. The tumor may be in one eye or in both eyes. Retinoblastoma rarely spreads from the eye to nearby tissue or other parts of the body. Retinoblastoma is usually found in only one eye and can usually be cured.

Types of retinoblastoma

Broadly, retinoblastoma is of two types:

• Heritable-- Retinoblastoma that is caused by an inherited gene mutation is called hereditary retinoblastoma. It usually occurs at a younger age than retinoblastoma that is not inherited. When hereditary retinoblastoma first occurs in only one eye, there is a chance it will develop later in the other eye. A child who has hereditary retinoblastoma is at risk for developing pineal tumors in the brain. This is called trilateral retinoblastoma.
• Non-heritable-- Retinoblastoma that occurs in only one eye is usually not inherited.

Symptoms

Possible symptoms of retinoblastoma include:

• Pupil of the eye appears white instead of red when light shines into it. This may be seen in flash photographs of the child.
• Eyes appear to be looking in different directions.
• Pain or redness in the eye.