Retinoblastoma: what is it?

Retinoblastoma (or RB) is a rare form of eye cancer that mainly affects children from birth to 5 years old.

This type of cancer affects 1 child in 15,000 births. Every year in Canada, 23 children are affected by this eye disease.

This condition results from a mutation of cells in the retina (retinoblasts) that grow disorderly. It can happen to only one eye (unilateral retinoblastoma) or to both eyes (bilateral retinoblastoma). Unilateral RB accounts for 75% of cases, while bilateral RB accounts for 25% of cases.

 

There are two types of retinoblastoma:

• Non-hereditary (or sporadic): it appears by chance, represents 60% of retinoblastomas and is mainly unilateral. The child will not pass it on to their own children as adults.
  
  
• Hereditary: represents about 40% of cases, can be familial or sporadic:
  
  
  • In the case of hereditary familial RB, one of the parents or another member of the family has already had retinoblastoma. It can be bilateral. The child will be at greater risk of developing other types of cancer throughout his life.
    
    
  • In the case of sporadic hereditary RB, no one has had retinoblastoma, but the mutated gene is passed on to the child.

 

Signs and symptoms

The parents notice it first.

The most common sign is a white effect in the pupil, visible especially when it is dilated (in dim light or under the effect of drops).

It also happens that one or both eyes squint (strabismus) because of the poor vision caused by retinoblastoma.

More rarely, redness or swelling of the eye may be observed.

All of these signs are not conclusive proof of retinoblastoma. However, it is important to quickly consult an optometrist to have the eyes checked if one or more of these symptoms appear.

 

Treatment

There may be surgery to remove the tumor.

If this operation is impossible, in some cases, it can lead to the removal of the eye.