Most cancers are named for the part of the body where the cancer starts. Retinoblastoma is a cancer that starts in the retina, the very back part of the eye. It is the most common type of eye cancer in children. 

Retinoblastoma is a rare disease. Only about 200 to 300 children are diagnosed with retinoblastoma each year in the United States. It is more common in infants and very young children than in older children. The average age of children when they are diagnosed is 2. It rarely occurs in children older than 6.

About 3 out of 4 children with retinoblastoma have a tumor in only one eye. In about 1 case in 4, both eyes are affected.

Retinoblastoma occurs about equally in boys and girls and in different races and ethnicities. It also occurs equally in the right or left eye.

Overall, more than 9 out of 10 children with retinoblastoma can be cured, but the outlook is not nearly as good if the cancer has spread outside of the eye.

The eyes develop very early as babies grow in the womb. During the early stages of development, the eyes have cells called retinoblasts that divide into new cells and fill the retina. At a certain point, the cells stop dividing and develop into mature retinal cells.

Rarely, something goes wrong with this process. Instead of developing into special cells that detect light, some retinoblasts continue to grow rapidly and out of control, and form a cancer known as retinoblastoma.

The chain of events that lead to retinoblastoma is complex, but it starts with an abnormality (mutation or change) in a gene called the retinoblastoma (Rb or RB1) gene. The normal RB1 gene helps keep cells from growing out of control. Depending on when and where the change in the RB1 gene occurs, 2 different types of retinoblastoma can result.

Cancer that forms in the tissues of the retina (the light-sensitive layers of nerve tissue at the back of the eye). Retinoblastoma usually occurs in children younger than 5 years. It may be hereditary or nonhereditary (sporadic).

Possible signs of retinoblastoma include "white pupil" and eye pain or redness.

These and other symptoms may be caused by retinoblastoma. Other conditions may cause the same symptoms. Check with a doctor if your child has any of the following problems:

  • 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.

Retinoblastoma and Enucleation

When Enucleation takes place due to Retinoblastoma, the immediate cooperation 

between Patient's parents , ophthalmologist, ocularist is needed.

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