Retinoblastoma treatment depends on the age of the child, whether one or both eyes are affected, and whether the cancer has spread to other parts of the body. Many of the treatments we offer can be performed on an outpatient basis.
Ophthalmic Artery Chemosurgery
Pioneered at Memorial Sloan Kettering, ophthalmic artery chemosurgery (OAC) is the standard of treatment at MSK for children with retinoblastoma. It is done on an outpatient basis and involves injecting chemotherapy drugs directly into the blood vessel that leads to the eye. First, the patient is given general anesthesia. Then, a thin tube is inserted through a blood vessel in the top part of the leg and threaded up to the eye artery. The chemotherapy is then injected into the eye. This method minimizes the body’s exposure to the drugs and reduces side effects. The average number of treatment sessions is about three for each eye, with each session delivered at four-week intervals. Further treatment with laser therapy, cryotherapy, radiation, and intraocular injections may also be required.
Laser therapy is a noninvasive, outpatient treatment for retinoblastoma. Lasers very effectively destroy smaller retinoblastoma tumors. This type of treatment is usually done by focusing light through the pupil onto and around the tumor. The light slowly heats up the tumor, destroying it.
Laser treatment is done under local or general anesthesia, usually does not cause any postoperative pain, and does not require any postoperative medications.
Cryotherapy freezes smaller retinoblastoma tumors. A pen-like probe is placed on the outer layer of the eyeball next to the tumor, and the tumor is frozen. It is done under local or general anesthesia on an outpatient basis. Cryotherapy may need to be repeated to successfully destroy all of the cancer cells.
Cryotherapy causes the eye and eyelids to swell for one to five days; sometimes they swell so much that the child can’t open his or her eyelids for a few days. This can be frightening, but it is usually harmless. Eye drops or ointment can reduce the swelling.
Intraocular chemotherapy involves injecting a tiny amount of chemotherapy into the eye. This type of chemotherapy is good for treating little fragments of tumor that break off and float in the jelly of the eye. This procedure is done on an outpatient basis while the patient is under anesthesia.
In this technique, a chemotherapy drug is given through a vein. The drug goes into the bloodstream, and as it passes through the eye, it shrinks the tumors. This process is also called chemoreduction. While it can be good at shrinking tumors, it rarely cures retinoblastoma on its own. At MSK, systemic chemotherapy with a single drug is used for small infants until they are big enough to have ophthalmic artery chemosurgery (at least three months of age and 15 pounds). This type of chemotherapy is given monthly on an outpatient basis. Our pediatric oncology team monitors the chemotherapy dosage and any side effects.
During an enucleation, the eye is surgically removed. Since OAC was developed, the need for enucleation has dramatically reduced. However, it is still a good treatment option for retinoblastoma that is too advanced for OAC. The procedure is done under general anesthesia. In the operation, the entire eye is removed, along with a piece of the optic nerve, and is sent to a pathologist for examination under a microscope.
The eyebrow, eyelids, and muscles of the eye are all left in place. Blinking, tearing, and movement of the brow are not affected by this surgery. The operation takes less than an hour and is not painful. Children go home the same day and are usually examined in the office next day, when postoperative instructions and care are explained.
A ball of plastic, rubber, or coral is placed where the eye had been so there is no cavity or hole. After the socket heals, it will look like the tissue on the inside of the lip.
The child is fitted for a prosthesis, or false eye, approximately four to six weeks after the operation. The prosthesis is made of plastic to look exactly like the other eye. There is currently no way to transplant or replace an entire eye.
Radioactive plaques are disks of radioactive material that were developed in the 1930s to radiate retinoblastoma. Today, iodine-125 or ruthenium-106 is used, and the plaques are custom built for each child.
The child must be hospitalized for this procedure. He or she undergoes two separate operations, one to insert the plaque and another to remove it, over three days. Long-term effects, including cataracts, bleeding and leaking of the retina, and impaired vision may occur.
External Beam Radiation Treatment
While rare today, external beam radiation has been used since the early 1900s as a way to save the eye and the child’s vision. Retinoblastoma is sensitive to radiation, and the treatment is often successful. The treatment is done on an outpatient basis five times per week over a three- to four-week stretch. However, long-term side effects and the advent of safer treatments like OAC have reduced the need for EBRT.
Treatment for Metastatic Retinoblastoma
Although it doesn’t often happen, retinoblastoma can spread (metastasize) to the brain, the central nervous system, and the bones. In these cases, doctors give systemic chemotherapy through the blood vessels or the brain for months or years after the diagnosis of metastatic disease.
The results have been very promising. A new medication delivered into the fluid that surrounds the brain and spinal cord is available for patients whose retinoblastoma has spread to the surface of the brain.
Protective eyewear is recommended for all children who have poor vision in one eye or whose eye has been removed. It should be worn during sports and other activities for life. In addition, sunglasses are always encouraged.
The majority of children with retinoblastoma in both eyes who are treated at MSK retain at least one eye with good vision, and many are able to retain the use of both eyes. However, all children with retinoblastoma in both eyes, and the 15 percent of patients with one affected eye who have the familial form of retinoblastoma, will be at much higher risk for other cancers not involving the eyes throughout their lives.
Follow-up appointments with an ophthalmologist and a pediatric oncologist are very important when a child is diagnosed with retinoblastoma. How often a child should go depends upon his or her age, whether an ophthalmologist suspects new tumors, whether one or both eyes are involved, and the type of treatment the child has received. At MSK, survivors of retinoblastoma are encouraged to get follow-up care at our long-term follow-up clinic.
Our social workers provide counseling, offer information and support, and can guide you to resources you may need. Child life specialists, teachers, and session and physician office assistants all work together to meet your child’s needs beyond treatment. Parents are encouraged to talk to their nurse and to call with questions between visits.