By: Tiara Memeh

What is Retinoblastoma?

Retinoblastoma is a rapidly developing cancer that develops from the immature cells of a retina, the light-detecting tissue of the eye and is the most common malignant tumor of the eye in children .

Retinoblastoma can be hereditary (passed down in families) or non-hereditary.

  • Forty percent of retinoblastoma patients have a genetic defect that leads to multiple tumors in one eye or both eyes. This is known as hereditary or germline retinoblastoma. These patients are typically diagnosed before 1 year of age.
  • Patients with hereditary retinoblastoma may pass this disease to their children.
  • Throughout their lives, patients with hereditary retinoblastoma are more likely to develop other cancers inside and outside of their eyes.
  • Sixty percent of patients have the nonhereditary form of retinoblastoma. Each of these patients develops a tumor in only one eye. Nonhereditary patients are diagnosed on average around 2 years of age.

Signs and Symptoms

Symptoms of retinoblastoma may include:

  • “Cat’s eye”: a white-yellow mass or glow seen through the pupil—often first noticed in a photo of a child’s face when the flash is used without “red eye reduction.” Normally, the center of the eye appears red in response to the camera flash, but in retinoblastoma, the center of the eye may have a white glow.
  • Poor vision
  • One or both eyes turning inward or outward
  • Pain from increased pressure in the eye as the tumor grows

How is Retinoblastoma treated?

Treatment of retinoblastoma depends on whether:

  • The tumor affects one eye or both eyes
  • The center of vision is affected by the tumor
  • The tumor has spread to other parts of the body

Treatment can include surgery, chemotherapy, focal therapy and radiation therapy.

  • Surgery— is used to remove the eye, if needed.
    • Eye(s) may be removed in children with advanced retinoblastoma.
    • In patients with only one affected eye: When that one eye is removed, more than 90 percent of those patients do not need any more treatment.
    • In patients with both eyes affected: If one eye is removed, treatment will focus on saving the remaining eye.