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Retinoblastoma is a malignancy of the retinal cell layer of the eye. It is the most common eye tumor in children and it usually occurs before the age of five. It can occur in one eye (unilateral) or in both eyes (bilateral). Retinoblastoma is usually confined to the eye and has not spread to other tissues. The present challenge for those who treat retinoblastoma is to prevent blindness and other serious effects of treatment that reduce the life span or the quality of life after treatment.

In about 40% of the cases, retinoblastoma is hereditary, or germline. The genetic locus responsible for a predisposition to retinoblastoma is located within the q14 band of chromosome 13.

Patients with retinoblastoma, particularly the hereditary type, have an increased frequency of second malignancies. These cancers are most often bone tumors, and they occur in up to 8% of retinoblastoma patients after 18 years of follow-up.

Treatment for Retinoblastoma

The treatment and the prognosis for retinoblastoma both depend on the spread, or stage, of the tumor.

Retinoblastoma is treated by surgery (enucleation), chemotherapy (vincristine, carboplatin, etoposide), cryotherapy, light coagulation, and radiation. The eye is spared whenever possible. Current statistics state an 80-90% 5 year survival rate.

Clinical trials for retinoblastoma can be found by searching the clinical trial database on the NCI web site. Follow these directions:

  • Start at the NCI clinical trials search page.
  • Choose type of cancer: “Retinoblastoma.”
  • You will be given more choices (e.g., type of cancer and type of trial). Choose the appropriate responses and click “search.”

Symptoms of Retinoblastoma

Symptoms can include:

  • whitish color behind the pupil
  • problems with eye movements (crossed eyes)
  • a red irritation that persists

The most common symptom is a whitish color behind the pupil, instead of the usual dark color. At well-baby check-ups, the pediatrician routinely checks your child’s eyes for this and other symptoms. As a parent, you should watch for the above symptoms between check-ups as well.

If you suspect that the abnormalities listed above are present and not adequately explained by your pediatrician, you should seek an eye examination by an ophthalmologist who specializes in pediatric eye disease. If the ophthalmologist detects abnormalities, an examination under anesthesia may be required to further evaluate your child.

Photos of the characteristic whitish color behind the pupil (and also good information on retinoblastoma) are:

Statistics, Risk Factors, Incidence

    • Accounts for about 3% of the cancers occurring in children younger than 15 years.
    • Most common in children under the age of 5.
    • 80-90% of children with retinoblastoma will be cured.

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