Retinoblastoma Facts

What is retinoblastoma?
Who gets retinoblastoma?
What causes retinoblastoma?
What are the survival rates for retinoblastoma?
How is retinoblastoma treated?

What is retinoblastoma?
Retinoblastoma is a cancer of the eye that develops in the cells of the retina.

Retinoblastoma, the most common malignancy of the eye in children, affects more than 300 children annually in the United States.

Retinoblastoma can affect one eye or both eyes at the same time.

Retinoblastoma is suspected when the child develops whitish discoloration of the pupil of the eye or when the eyes are crossed or misaligned.

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Who gets retinoblastoma?
Retinoblastoma usually occurs in children from birth to 5 years of age.

It is rare beyond the age of 6.

Retinoblastoma is a world problem affecting approximately 8,200 children each year. It is estimated that 92 percent of the world’s children have minimal access to optimal care.

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What causes retinoblastoma?
This disease results from specific genetic defects in one of the chromosomes in the cell.

Approximately 30 to 40 percent of retinoblastoma cases are hereditary.

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.

A child should be monitored from birth if one parent had retinoblastoma. The parent may have passed on the genetic mutation to the child.

Any child can get retinoblastoma regardless of their sex, age or ethnic background.

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What are the survival rates for retinoblastoma?
Nearly 95 percent of children with retinoblastoma are cured, but they may lose an eye.

Attempts to preserve the eye involve treatments, which can result in significant side effects.

When retinoblastoma spreads beyond the eye, a child usually has a very poor outcome.

Early detection of retinoblastoma is extremely important or the disease can be fatal.

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How is retinoblastoma treated?
When one eye is involved and the disease is advanced, the eye is usually surgically removed. This treatment results in the loss of an eye, but a permanent cure for more than 90 percent of patients.

If both eyes are involved, then chemotherapy, consisting of anti-tumor drugs, is used first to shrink the tumors in both eyes.

Ophthalmologists may treat small tumors by using laser therapy or a freezing treatment called cryotherapy.

Other novel approaches, such as gene therapy and proton therapy, are being used in clinical trials to treat retinoblastoma patients, showing promising outcomes.

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