Treatments for extraocular retinoblastoma

Extraocular retinoblastoma is retinoblastoma that has spread from the eye to tissues around the eye, and to the brain and spinal cord (called the central nervous system, or CNS) or to other parts of the body.

The following are treatment options for extraocular retinoblastoma and recurrent extraocular retinoblastoma. The healthcare team will suggest treatments based on your child’s needs and work with you to develop a treatment plan. Depending on where the retinoblastoma has spread, your child may have one or more of these treatments.

Chemotherapy uses drugs to destroy cancer cells. The chemotherapy used for extraocular retinoblastoma may be systemic or regional.  It may be given alone or with other treatments, such as radiation therapy. It may also be used before or after surgery.

Intrathecal chemotherapy is given to the brain and spinal cord (called the central nervous system, or CNS) through a lumbar puncture into the cerebrospinal fluid (CSF). It may be used to treat extraocular retinoblastoma that has spread to the brain.

High-dose chemotherapy with stem cell rescue may be offered for extraocular retinoblastoma in the brain or spinal cord, or for cancer that has spread to other distant sites.

Find out more about chemotherapy for retinoblastoma.

Enucleation is surgery to remove the eye. It may be offered to treat extraocular retinoblastoma.

Find out more about surgery for retinoblastoma.

Radiation therapy uses high-energy rays or particles to destroy cancer cells.

External radiation therapy may be used to treat extraocular retinoblastoma and recurrent extraocular retinoblastoma. If retinoblastoma has reached the CNS, radiation may be given to the brain and spine as well (called craniospinal radiation therapy).

Radiation therapy may be given along with chemotherapy or after surgery.

Find out more about radiation therapy for retinoblastoma.

Children with cancer may be treated in a clinical trial. Clinical trials look at new ways to prevent, find and treat cancer. Find out more about clinical trials.

Follow-up after treatment is an important part of cancer care. Children will need to have regular follow-up visits, especially in the first 5 years after treatment has finished. These visits allow the healthcare team to follow your childʼs progress and recovery from treatment, and to watch for recurrence. Find out more about follow-up care, life after treatment and long-term survivorship.

Treatment is successful for many children with cancer, but in some cases it isn’t. When cancer is advanced, the focus of treatment can change from cure to quality of life. There is help and support available. Find out more about when a cure is not possible.