Treatment

Clinical trials are the standard of care for the treatment of children with cancer. In fact, more than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare the standard treatments (the best treatments available) with newer treatments that may be more effective. Cancer in children is rare, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. Investigating new treatments involves careful monitoring using scientific methods and all participants are followed closely to track progress.

To take advantage of these newer treatments, all children with cancer should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children with cancer and have access to the latest research. Many times, a team of doctors treats a child with cancer, including pediatric, oncologic, and radiation specialists. Pediatric cancer centers often have extra support services for children and their families, such as nutritionists, social workers, and counselors. Special activities for children with cancer may also be available.

Several types of therapies are used for retinoblastoma, and more than 90% of children can be cured. In addition to cure, an important goal of therapy for retinoblastoma is the preservation of vision. Many of these treatment approaches have become available as a result of clinical trials. The Children’s Oncology Group has recently developed treatment clinical trials that include retinoblastoma.

Treatments for retinoblastoma include:

Surgery

Surgery to remove the eye is called enucleation. Children with a tumor in one eye only can often be cured with this treatment. In children with a tumor in both eyes, enucleation is used only if the ocular oncologist (a doctor who specializes in eye cancer) determines that preserving vision using other treatment is not possible. Surgical treatment for retinoblastoma should be performed by specially trained pediatric ophthalmologists.

If enucleation is the best treatment to cure the cancer, there will be some visual loss. This may result in trouble with depth perception, but most children adapt well to these differences.

Many people worry about what they will look like when they have an eye removed. The cosmetic surgery available today usually yields good cosmetic results. To fill the area left by the missing eye, the person is fitted for a prosthesis (artificial eye). The prosthesis will look and behave almost the same as a natural eye. For example, the artificial eye will move along with the person’s remaining eye, just not as much as a natural eye moves. Family members may be able to tell that the eye is not real, but it is unlikely that strangers will know. If enucleation is required, talk with your doctor about a prosthesis; it may take many weeks for patients to receive the prosthesis.

Radiation therapy

Radiation therapy uses high-energy x-rays or other particles to kill cancer cells. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation therapy given from a machine outside the body. Radioactive plaque therapy, also called internal radiation therapy or brachytherapy, is the delivery of radiation therapy directly to the eye with a disc containing radiation.

Fatigue, drowsiness, nausea, vomiting, and headache are common temporary side effects of radiation therapy. Radiation therapy in young children can interfere with normal growth, including growth of the orbital bones (bones around the eye), depending on the dose. The increased risk of additional tumors later in life for children with the hereditary form of retinoblastoma is further increased following external-beam radiation therapy. These effects are not seen after radioactive plaque therapy.

Cryotherapy

Cryosurgery, also called cryotherapy or cryoablation, uses liquid nitrogen to freeze and kill cells. More than one freezing may be needed.

Laser therapy

Laser therapy uses heat in the form of a laser to shrink smaller tumors. It may be called thermotherapy (or TTT for transpupillary thermotherapy), and it may be used alone or in addition to cryotherapy or radiation therapy. Photocoagulation is a different type of laser therapy that uses light to shrink tumors.

Chemotherapy

Chemotherapy uses drugs to kill cancer cells, and may be used to shrink tumors in the eye. It is given by a pediatric oncologist and often makes it possible to completely eliminate any remaining smaller tumors with the following focal (localized) measures:

• Thermotherapy or photocoagulation (laser therapy)
  
  
• Cryotherapy
  
  
• Radioactive plaque therapy

Chemoreduction is a treatment approach that is often used in children with bilateral disease (in both eyes) in the hope of avoiding enucleation and preserving vision in at least one eye. The ophthalmologist, in consultation with the pediatric oncologist, will determine if this treatment is appropriate. Both doctors will monitor the response to treatment regularly and may recommend additional treatment to prevent the cancer from returning.

The drugs used most often are vincristine (Oncovin), carboplatin (Paraplatin), and etoposide (VePesid, Etopophos, Toposar). Depending on the extent of the tumor, a combination of two or more drugs will be recommended. All chemotherapy has side effects that occur during the period of treatment. Some drugs have the potential for specific long-term side effects. Talk with your doctor about the specific drugs used before treatment begins.

The medications used to treat cancer are continually being evaluated. Talking with your child’s doctor is often the best way to learn about the medications they've been prescribed, their purpose, and their potential side effects or interactions with other medications. Learn more about your child’s prescriptions through Cancer.Net's Drug Information Resources, which provides links to searchable drug databases.

Recurrent retinoblastoma

If retinoblastoma returns after treatment, the treatment plan depends on where the cancer recurred and how aggressive the new tumor is. The doctor may recommend surgery, radiation therapy, chemotherapy, and/or focal measures (photocoagulation, thermotherapy, or cryotherapy).

To learn about the terms used in this section, read the Cancer.Net Feature: Cancer Terms to Know: During Treatment.