About

Retinoblastoma in Children


About Retinoblastoma

Although retinoblastoma is the most common type of pediatric eye cancer, it is still rare. It is typically diagnosed in infants and young children around two years old, and it occurs equally in girls and boys. Diagnosis in children over six years old is uncommon.

Approximately three out of four children with retinoblastoma suffer from unilateral retinoblastoma, in which there is a tumor in only one eye. Bilateral retinoblastoma refers to cases in which both eyes are affected. More than nine out of ten children with retinoblastoma in the US are cured, but the prognosis is less optimistic if this eye cancer has spread to other tissues or organs.


The eyes begin to develop in utero, when cells called retinoblasts rapidly multiply to create new cells for the retina. After this early stage of the eye’s development, retinoblasts cease to reproduce and instead grow into mature retinal cells. When this process goes wrong and the retinoblasts grow out of control, the result is retinoblastoma.

Usually, the course of events that leads to retinoblastoma starts with a mutation in the RB1 gene, which is the gene that prevents cells from growing uncontrollably.

When left untreated, retinoblastoma tumors can grow to fill most of the eyeball. If cells break away from the main tumor on the retina, they can form additional tumors on other parts of the eye. Over time, retinoblastoma can also spread beyond the eyeball to lymph nodes and other organs. However, the vast majority of cases are detected and treated when retinoblastoma is present only in the eyeball.

Very few known risk factors exist for retinoblastoma, but some possible factors include:

  • Age: Most diagnoses occur before the child is three years old, and rarely after age six
  • Heredity: Having a parent who had congenital retinoblastoma; however, retinoblastoma may also occur in a child without any family history of the cancer
  • Parental lifestyle: Exposure to chemicals in diesel or gasoline exhaust during pregnancy; the mother not eating enough fruits and vegetables during pregnancy; exposure of fathers to radiation; older age of the father

In general, the symptoms of retinoblastoma in children present before age two, and they are noticed most frequently by a parent or doctor. The most common signs include:

Leukocoria: Also called “white pupillary reflex,” this is the most typical symptom of retinoblastoma; it refers to when the eye’s pupil looks white when light shines into it
(instead of red because of blood vessels at the back of the eye)

Strabismus: A problem with eye teaming, also called “lazy eye”

Nystagmus: Involuntary eye movements

Redness of the white part of the eye or around the eye

Different color in each iris

Weak vision or a change in vision

Eye pain

Retinoblastoma is classified into three main groups, based on the stage:

  1. Intraocular retinoblastoma: The cancer is limited to the eye
  2. Extraocular retinoblastoma: The cancer has spread beyond the eye. Orbital retinoblastomas refer to when cancer has only moved into the eye socket, versus metastatic retinoblastomas, which have spread to other distant organs
  3. Recurrent retinoblastoma: The cancer has returned – either to the eye or in other body parts

The stage of retinoblastoma is strongly associated with the prognosis for children who have this cancer. Staging is also significant for designing the most effective treatment program.


What is Retinoblastoma?

The eyes begin to develop in utero, when cells called retinoblasts rapidly multiply to create new cells for the retina. After this early stage of the eye’s development, retinoblasts cease to reproduce and instead grow into mature retinal cells. When this process goes wrong and the retinoblasts grow out of control, the result is retinoblastoma.

Usually, the course of events that leads to retinoblastoma starts with a mutation in the RB1 gene, which is the gene that prevents cells from growing uncontrollably.

When left untreated, retinoblastoma tumors can grow to fill most of the eyeball. If cells break away from the main tumor on the retina, they can form additional tumors on other parts of the eye. Over time, retinoblastoma can also spread beyond the eyeball to lymph nodes and other organs. However, the vast majority of cases are detected and treated when retinoblastoma is present only in the eyeball.

 

What are the risk factors for retinoblastoma?

Very few known risk factors exist for retinoblastoma, but some possible factors include:

  • Age: Most diagnoses occur before the child is three years old, and rarely after age six
  • Heredity: Having a parent who had congenital retinoblastoma; however, retinoblastoma may also occur in a child without any family history of the cancer
  • Parental lifestyle: Exposure to chemicals in diesel or gasoline exhaust during pregnancy; the mother not eating enough fruits and vegetables during pregnancy; exposure of fathers to radiation; older age of the father

 

What are the symptoms of retinoblastoma?

In general, the symptoms of retinoblastoma in children present before age two, and they are noticed most frequently by a parent or doctor. The most common signs include:

  • Leukocoria: Also called “white pupillary reflex,” this is the most typical symptom of retinoblastoma; it refers to when the eye’s pupil looks white when light shines into it
    (instead of red because of blood vessels at the back of the eye)
  • Strabismus: A problem with eye teaming, also called “lazy eye”
  • Nystagmus: Involuntary eye movements
  • Redness of the white part of the eye or around the eye
  • Different color in each iris
  • Weak vision or a change in vision
  • Eye pain

 

What are the stages of retinoblastoma?

Retinoblastoma is classified into three main groups, based on the stage:

    1. Intraocular retinoblastoma: The cancer is limited to the eye
    2. Extraocular retinoblastoma: The cancer has spread beyond the eye. Orbital retinoblastomas refer to when cancer has only moved into the eye socket, versus metastatic retinoblastomas, which have spread to other distant organs
    3. Recurrent retinoblastoma: The cancer has returned – either to the eye or in other body parts

The stage of retinoblastoma is strongly associated with the prognosis for children who have this cancer. Staging is also significant for designing the most effective treatment program.

 

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