Retinoblastoma

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Treating Retinoblastoma TOPICS

Treatment of retinoblastoma, based on extent of the disease

A number of factors can affect the treatment options your child’s doctor recommends. Some of these include:

  • Whether tumors are in one or both eyes
  • The size and location of the tumor in the eye(s)
  • The chance for saving vision in the eye(s)
  • Whether the tumor is still confined within the eye(s) or has spread elsewhere

A very important factor is whether the retinoblastoma has affected one or both eyes. Treatment is different depending on whether it affects one eye or both.

For children with only one eye affected, treatment depends on whether vision in the eye can be saved. If the chance to save vision is poor, the treatment is often surgery to remove the eye. Surgery was used more often in the past, as it offered the best chance to ensure all of the cancer was removed. But in recent years, doctors have become more comfortable with using other treatments (often chemotherapy plus some form of local treatment) if there is a good chance of saving vision in the eye.

If the retinoblastoma is in both eyes, doctors will try to save at least one eye if at all possible so that the child maintains some vision. Most children with retinoblastoma in both eyes will be treated first with chemotherapy to shrink the tumors (called chemoreduction), followed by some form of local treatment and possibly radiation therapy. Surgery (removal of the eye) is reserved for the most advanced tumors.

Many children will receive several types of treatment. Treatments over months or years may be needed, especially in eyes treated with cryotherapy and/or photocoagulation after chemotherapy.

No matter which types of treatment are used, it is very important that they are given by experts at centers experienced in treating these tumors.

If the eye can see and probably can be saved

For some smaller tumors, local treatments such as laser therapy or cryotherapy may be the only treatment needed.

More often, tumors are larger or in hard-to-treat areas. Treatment usually includes a combination of chemotherapy and focal treatments. If systemic chemotherapy (chemotherapy given by vein) is used, it is given for about 6 months to shrink the tumor as much as possible. More recently, some doctors have begun to give chemotherapy directly into the artery that feeds the eye (intra-arterial chemotherapy) instead of systemic chemotherapy. Usually a few treatments are needed, each given a few weeks apart.

Depending on how much the tumor shrinks and where it is in the eye, different focal treatments can then be applied, usually starting after the first or second cycle of chemotherapy. These treatments may include brachytherapy (plaque radiotherapy), cryotherapy, laser therapy, or thermotherapy. External radiation therapy may also be needed, but it is usually delayed until the end of chemotherapy.

If the combination of these treatments doesn’t control the disease, surgery to remove the eye may be needed.

If the eye cannot see or cannot be saved

If there is no vision in the eye, if the tumor is so advanced within the eye that there is no hope of cure by other means, or if there is painful glaucoma, then surgery is done to remove the eye and place an orbital implant in the socket.

If the disease affects only one eye, no other treatments may be required. But sometimes, after looking at the removed eye under the microscope, the doctors find that some retinoblastoma cells may have escaped the eye and thus may come back later in other parts of the body. These children may be given chemotherapy, possibly along with radiation therapy to the area around the eye, to try to lower this risk.

In some instances where there are large tumors in both eyes, chemotherapy may be used first to try to shrink the tumors and avoid the need for surgery in both eyes. If chemotherapy shrinks the tumors enough, local therapies such as brachytherapy (plaque radiotherapy), cryotherapy, laser therapy, or thermotherapy may allow at least one eye (and some vision) to be saved.

Some children with retinoblastoma in only one eye may actually have the hereditary form of retinoblastoma, which means they will probably develop disease in the other eye as well. Therefore, it is very important that children with retinoblastoma in one eye continue to have the other eye examined regularly after treatment.

If the cancer has spread outside the eye

In this uncommon situation, treatment is usually a combination of chemotherapy, radiation, and in some cases surgery.

If the cancer has spread only to the orbit (the area around the eye), treatment with chemotherapy, surgical removal (enucleation) of the eye, and radiation therapy to the orbit is often successful.

If the cancer has spread outside the orbit to distant parts of the body such as the liver or the bones and bone marrow, the chances of a cure using standard chemotherapy and other treatments are very low. In these cases, using higher doses of chemotherapy followed by a stem cell transplant can often be successful.

Cancers that have spread to the brain (including trilateral retinoblastomas, which are retinoblastomas that usually start in the pineal gland) are very hard to treat, and the chances of a cure using standard treatments are low. High-dose chemotherapy and stem cell transplant has shown some promise for these cancers in a recent small study. Clinical trials using other newer treatments may be an option in this situation as well.

If the cancer comes back in the eye after initial treatment

Treatment of cancer that recurs in the eye depends on the size and location of the tumor and on what treatments were used the first time. If the tumor is small, the child’s sight can often be saved while the cancer is destroyed with local treatments such as cryotherapy, laser therapy, radiation therapy (if not already used), or other treatments. Chemotherapy may be given first. If the child’s sight cannot be saved, the eye may need to be removed. Either way, the chance of a cure is very good as long as the cancer is still confined to the eye.

If the cancer comes back outside the eye after initial treatment

Cancers that recur outside the eye are harder to treat. Options may include chemotherapy and radiation, or high-dose chemotherapy with a stem cell transplant in some cases. In this situation, the treatment and the chances of cure are similar to what is described above (when the cancer has spread outside the eye before initial treatment).

Summary

If the cancer is in only one eye and the potential for saving sight is good, chemotherapy (either by vein or directly into the artery of the eye) and local treatments such as cryotherapy laser therapy, thermotherapy, and plaque radiation are used (although very small tumors, which are very rare, can be treated with local treatments alone). Otherwise the eye will likely need to be removed.

If the cancer is in both eyes, then the doctors will try to save as much vision as possible. The treatment usually starts with chemotherapy, followed by local treatments.

In all cases, children who have had retinoblastoma need to be followed closely for some time after treatment.


Last Medical Review: 12/05/2013
Last Revised: 12/05/2013