Retinoblastoma Treatment

New Patient Appointment or 214-645-2020

The priorities for treating retinoblastoma are first, to save the child’s life; second, to preserve the eye if possible; and third, to save as much vision as possible.

When retinoblastoma is diagnosed and treated promptly, over 95% of children with the disease in the United States can be saved. Most children can be successfully treated without removal of the eye, and many retain useful vision.

UT Southwestern ocular oncologists use the entire range of leading-edge treatments for retinoblastoma. The treatment plan for each patient is individually designed to account for numerous factors, including:

Tumor number, size, and location
Tumor seeding, retinal detachment, bleeding, glaucoma, and tissue invasion
Involvement of one or both eyes
Spread of the cancer outside of the eye
The general health of the child
The family’s preferences

Treatment options include:

Laser Therapy

A laser emits certain wavelengths of light, some of which can be used to destroy cancer cells. Laser therapy is especially useful for treating small tumors near sensitive structures of the eye.

Cryotherapy

This is a freezing treatment that employs a small probe with a very cold tip to directly kill cancer cells. Cryotherapy is less commonly used than laser therapy but can be useful for treating slightly larger tumors near the front part of the retina.

Chemotherapy

For many years, chemotherapy has been used to shrink retinoblastomas, but the tumors invariably grow back without further treatment. In the 1990s, research showed that chemotherapy could be successful if followed up by laser or cryotherapy to kill off any remaining cancer cells after tumor shrinkage. Since then, there have been continued improvements in the use of chemotherapy for successfully treating retinoblastoma and avoiding eye removal.

Methods of delivering chemotherapy that are commonly used for retinoblastoma include:

Systemic (intravenous) chemotherapy

This is the traditional method of chemotherapy in which the medications are delivered through a vein and travel throughout the entire body. Systemic chemotherapy is very effective for retinoblastoma tumors through Group C but is less effective in Groups D and E. Most children require at least six cycles of chemotherapy at about one-month intervals. Following each cycle of chemotherapy, the child returns to the ocular oncologist for another examination under anesthesia to document and measure each tumor for evidence of treatment response and to treat each tumor with laser or cryotherapy. The goal of this so-called consolidative therapy is to eliminate any remaining cancer cells that were not killed by the chemotherapy.

UT Southwestern pediatric oncologists are highly experienced in treating young children with retinoblastoma, and they work closely with our ocular oncologists to coordinate the care of each patient.

Intra-arterial chemotherapy (IAC)

Intra-arterial chemotherapy (IAC) is a method for directly delivering chemotherapy to the eye. IAC has revolutionized the treatment of retinoblastoma and increased our ability to save eyes and reduce side effects. IAC is a specialized procedure that is performed by a highly trained pediatric endovascular neurosurgeon, who introduces a tiny tube (catheter) into a larger artery in the child’s body, usually the femoral artery in the upper thigh. The catheter is then carefully threaded up through various arteries until it reaches the ophthalmic artery, which supplies blood to the eye.

IAC is performed in a specialized interventional radiology suite, and it can be used to treat one or both eyes during the same treatment session. The number of sessions of IAC varies according to the agents used, tumor response, extent of disease, and other factors. The main contraindication to IAC is lack of access to the ophthalmic artery, either because the femoral artery cannot be safely catheterized, or the arteries around the eyeball have an unusual arrangement that prevents the catheter from passing through to the eye. Arterial access is difficult in very small infants, so IAC is generally not performed on babies weighing less than 16-18 pounds (7-8 kilograms). IAC is highly effective in treating retinoblastoma through Group D, and it can save the eyes of a significant percentage of Group E patients, all of whom previously would have undergone enucleation (removal of the eye).

UT Southwestern and Children’s Medical Center have created the only multidisciplinary retinoblastoma program in North Texas that offers intra-arterial chemotherapy for children with retinoblastoma. Our ocular oncology team is among the most experienced in the United States in the use of this specialized cancer treatment.

Intravitreal chemotherapy

This procedure is performed by the ocular oncologist during the examination and while the patient is under anesthesia. It is most often used as a supplement to systemic or intra-arterial chemotherapy for treating vitreous tumor seeds. Vitreous seeding was once the most common cause of treatment failure in eyes receiving systemic or intra-arterial chemotherapy, but with the development of intravitreal chemotherapy, most of these eyes can now be saved. The procedure is performed under sterile conditions and takes about two minutes. The chemotherapy is prepared and delivered to the operating room, where a tiny amount is drawn into a syringe with a very thin, short needle. The surgeon inserts the needle into a specific part of the eye while simultaneously performing cryotherapy to prevent any cancer cells from escaping through the needle track. UT Southwestern ocular oncologists are among the most experienced with this procedure in the United States and have excellent outcomes.

Radiation Therapy

External beam radiation therapy (EBRT), in which a large machine outside the body delivers high-powered beams of radiation, was commonly used for treating retinoblastoma until the 1990s. However, EBRT has been largely replaced by chemotherapy due to the concerns of secondary cancers, arrested bone growth, and other side effects of radiation. Currently, EBRT is used only in very rare circumstances to save certain eyes for which other therapies have failed.

Another form of radiation therapy is called plaque brachytherapy, a surgical procedure in which a small disclike plaque made of gold and carrying tiny radioactive pellets is temporarily attached to the outside of the eye near the tumor. Plaque brachytherapy has fewer side effects than EBRT and can be highly effective for treating select tumors that have not begun to shed tumor seeds and are too large to treat with laser or cryotherapy but small enough to be covered by the plaque.

Surgery

Despite the availability of advanced treatment options, surgical removal of the eye (enucleation) is still the safest option for some children.

Enucleation is usually recommended when the cancer fills most of the eye, invades the optic nerve or other structures, causes bleeding or high pressure (glaucoma) inside the eye, or otherwise creates a danger of cancer spreading if the eye is not removed.

Enucleation is performed under general anesthesia by an ophthalmic surgeon skilled in performing such procedures in young children. The surgeons will disconnect the eyeball from the muscles that control eye movement and from the optic nerve. They will then insert a ball-shaped implant into the eye socket and attach it to the muscles, so the implant will move with the other eye. Children usually recover quickly from this procedure.

About six weeks after surgery, a customized prosthesis (artificial eye) will be made by an ocularist to match the color and appearance of the remaining natural eye. The prosthesis is a shell-like device that fits over the eye socket implant that was placed at surgery. The prosthesis needs to be checked and cleaned periodically by the ocularist and may need to be replaced every few years, especially as the child grows.