Retinoblastoma

The Texas Children’s Cancer Center’s Retinoblastoma Center of Houston and team members from the Brain Tumor Program treat patients with retinoblastoma.

What is retinoblastoma?

Retinoblastoma is disease in which cancer cells are found in the retina, which is a thin membrane on the back of the inside of the eye. It is curable in most cases.

Retinoblastoma is not very common. It occurs mostly in children less than five years old. Retinoblastoma may be found in one eye or in both eyes. Some children have retinoblastoma that runs in families (hereditary retinoblastoma). Brothers and sisters of children with retinoblastoma should be checked by an eye doctor, or should have laboratory tests to find out whether they are at risk for developing the disease. If retinoblastoma occurs in one eye, it may not be hereditary. If it is in both eyes, it is always hereditary.


What is the treatment for retinoblastoma?

Treatment for retinoblastoma depends on the stage of the disease and the age and general health of the child being treated.

Surgery is a common treatment for retinoblastoma.

  • A surgical procedure called enucleation removed the entire eye. A child may have normal vision in the eye that is not removed.
  • Cryosurgery kills the cancer cells by freezing them. This procedure is used for tumors that are very small.
  • Photocoagulation uses a narrow beam of very strong light to kill blood vessels that feed the tumor caused by the cancer.
  • Radiation therapy can also be used to treat retinoblastoma. Radiation therapy uses high-energy x-rays to kill cancer cells and shrink tumors.

Chemotherapy uses drugs to kill cancer cells. With systemic chemotherapy, chemotherapy drugs enter the bloodstream and travel through the body. These drugs can kill cancer cells outside the eye. If cancer cells are found in the fluid that surrounds the brain and spinal cord, chemotherapy drugs may be put into the fluid surrounding the spinal cord using a needle. This is called intrathecal chemotherapy. Radiation and/or chemotherapy may be used depending on the spread of the tumor beyond the eye.

Stages and Treatment

Tumors are found in one eye or in both eyes, but have not spread to tissues around the eye.

Treatment

Your child’s treatment depends on whether the cancer is in one eye of both eyes. If the cancer is in one eye, your child’s treatment may include one or more of the following:

  • surgery to remove the eye (enucleation)
  • external beam radiation therapy
  • photocoagulation, with or without external beam radiation therapy
  • cryosurgery

If the cancer is in both eyes, your child’s treatment may include one or more of the following:

  • surgery to remove the eye with the most cancer, followed by radiation therapy to the other eye
  • radiation therapy to both eyes
  • chemotherapy initially, followed by radiation therapy
  • Extraocular retinoblastoma

Retinoblastoma cells have spread to tissues around the eye, or to other parts of the body.

Treatment

Your child’s treatment may include one or all of the following:

  • Radiation therapy to the eye
  • Intrathecal chemotherapy
  • Systemic chemotherapy
  • Recurrent retinoblastoma

Recurrent disease means the cancer cells have come back after treatment.

Treatment

Your child’s treatment depends on where the cancer came back. If it came back only in the eye, your child may have surgery, photocoagulation, cryosurgery or radiation therapy. If the cancer came back in another part of the body, your child may receive chemotherapy or radiation therapy.


More Information about Retinoblastoma

  • Retinoblastoma is the most common malignancy of the eye in children
  • It 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 not aligned.
  • Most often there is no parental history of eye cancer in childhood.
  • This disease results from specific genetic defects in one of the chromosomes in the cell.
  • Retinoblastoma can be hereditary.
  • If one parent had retinoblastoma (usually in both eyes as a child) and then passed on the genetic mutation to the child, then the child can develop retinoblastoma and should be monitored closely from birth.
  • Retinoblastoma usually occurs in children less than 3 years of age. It is very rare beyond the age of 6.
  • Any child can get retinoblastoma regardless of their sex or age.
  • Almost 90 percent of children with retinoblastomas are cured. When retinoblastoma spreads beyond the eye, a child usually has a very poor outcome.
  • Early detection of retinoblastoma is extremely important.
  • In a child with one eye involved, the disease is usually advanced. In this situation, the eye is surgically removed, which results in the loss of the eye and in more than 90 percent of patients, a permanent cure.
  • If both eyes are involved, then chemotherapy, consisting of anti-tumor drugs is used first to shrink the tumors in both eyes. Eventually, the eye that is most affected is removed and the other eye may receive radiation treatment.
  • Ophthalmologists also treat small tumors if necessary by using laser therapy or cryotherapy.

 


What is Texas Children’s Cancer Center doing to study and treat retinoblastoma?

A novel approach to treating retinoblastoma was developed at Texas Children’s Cancer Center. The ultimate goal of this treatment is to cure the disease, but also save vision. This treatment involves injecting viral particles carrying an enzyme into the tumor. The tumor cells take up these viral particles. When these tumor cells are then exposed to an antiviral drug, the tumor cells are destroyed. The first such clinical trial was performed at Texas Children’s.