Retinoblastoma

What are the signs and symptoms of retinoblastoma?

Common signs and symptoms of retinoblastoma include:

• Leukocoria (a white pupil)
• Change in pupil size or eye bulging
• Worsening vision in one or both eyes over weeks/months
• The development of strabismus (eyes crossing or drifting) over weeks/months

What tests are used to diagnose retinoblastoma?

Retinoblastoma is typically a clinical diagnosis based on the appearance of the tumor in the eye. After a thorough dilated eye exam by an ophthalmologist, patients typically undergo the following tests to help confirm the diagnosis, determine how advanced the disease is, and prepare for the appropriate treatment course:

• Eye exam under anesthesia by an ophthalmologist to gather additional information on the appearance of the tumor(s) and obtain baseline photographs
• Ultrasound of the eye
• MRI and angiogram of the eyes and brain (under sedation)
• Blood tests

How is retinoblastoma treated?

Based on the results of the initial exams and tests, the eye(s) will be classified into a group or stage that helps guide treatment decisions.

A team of ophthalmologists, oncologists, and radiologists work together to determine the best treatment plan for each patient based on whether the tumor involves one or both eyes as well as their staging.

Treatment options include:

• Chemotherapy is the most common treatment for larger tumors or tumors that affect both eyes. Administration of chemotherapy can come in various forms:
  • Systemic chemotherapy is medication that is administered through an IV or a port placed in a separate procedure for improved IV access
  • Intra-arterial chemotherapy is medication that is delivered under sedation to the eye via the artery that supplies blood to the eye by a radiologist under imaging guidance
  • Intravitreal chemotherapy is medication that is injected directly into the eye using a small caliber needle under sedation
• Laser treatment or cryotherapy treatment (freezing the tumor) can be used for small tumors
• Enucleation is the surgical removal of the eye and is only recommended for large tumors or tumors that have progressed despite previous attempts to reduce the risk of tumor spread beyond the eye

Our ophthalmologists follow patients closely to monitor their response to treatment as well as for any future recurrence or complications. These follow-up exams are frequent at first (every 3 to 6 weeks) and typically take place under sedation if your child is young. Eventually, patients will transition to clinic visits with reduced frequency as the disease remains in remission and the child is able to participate in exams in the office.

Because of advances in diagnosis and treatment of retinoblastoma, more than 95% of children in the United States treated for this disease are cured of the cancer, and more than 90% of patients retain normal vision in at least one eye.

Why choose us for treatment of retinoblastoma?

Children’s Colorado has a multidisciplinary team of experts that can quickly diagnose and treat children with retinoblastoma. Our Retinoblastoma Program combines specialists from different areas of expertise that work as a team to develop the most effective treatment plan. This is important because the quicker your child’s doctor can diagnose and treat retinoblastoma, the better chances they have of preserving your child’s vision.

Contact us by calling the Department of Pediatric Ophthalmology at 720-777-2020 or the Center for Cancer and Blood Disorders at 720-777-6740.

Helpful resources

The American Association for Pediatric Ophthalmology and Strabismus (AAPOS) works to advance the quality of children's eye care, support the training of pediatric ophthalmologists, support research activities in pediatric ophthalmology and advance the treatment for conditions like retinoblastoma.