We understand how difficult a diagnosis of retinoblastoma can be, both for your child and for your whole family. That's why our physicians are focused on family-centered care: From your first visit, you'll work with a team of professionals who are committed to supporting all of your family's physical and psychosocial needs. We'll work with you to create a care plan that's best for your child.
The good news is that since it's usually found before it spreads outside of the sclera (white part of the eye), retinoblastoma is highly curable. And there are many types of treatment that can save sight in the affected eye.
Some treatments are standard and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Until recently, chemotherapy was used only to treat retinoblastoma that had spread outside the eye. Now, more doctors are using chemotherapy as a first-line of treatment in order to prevent the spread of cancer to other parts of the body.
At Boston Children's Hospital, we use the following types of chemotherapy treatment:
Chemoreduction—medicine is given through an IV, passes through your child's blood stream, and if successful, causes the tumors to shrink within a few weeks. Depending on which drugs your doctor prescribes, your child may or may not be hospitalized during treatment.
Afterwards, we'll re-examine your child, and treat any remaining tumors with cryotherapy, thermotherapy, photocoagulation or radiation. Chemoreduction alone rarely if ever cures retinoblastoma, and some children may require as many as twenty treatments every three weeks.
Intra-arterial chemotherapy—a newer treatment for more advanced retinoblastoma in which the medicine is injected directly into the main blood vessel of the eye (ophthalmic artery). This treatment was designed to minimize the amount of contact the chemotherapy has with the rest of your child's body, and reduce side effects. It can deliver a much higher dose of chemo straight to the eye, and may kill more cancer cells in fewer treatments.
During this procedure, your child is put to sleep by an anesthesiologist. Then the surgeon inserts a thin tube through a blood vessel in your child's groin, and feeds it up to the ophthalmic artery. Then the medication is injected through the tube up to the eye.
The average number of treatment sessions is about three for each eye, delivered every four weeks. Download fact sheet for referring physicians
Surgery is usually reserved for children with advanced retinoblastoma that doesn't respond to other treatments. It's most often a last resort used when the health of the child is in serious jeopardy. In these cases, the eye may need to be removed, during a procedure called enucleation. While it's technically possible for the tumor to return following this procedure, it's extremely rare.
Enucleation is a relatively simple operation that's performed while your child is under general anesthesia. Following the surgery, the eye is replaced with an orbital implant, and after the eye has healed (usually around three months), an artificial eye—that can be made to match your child's healthy eye—can be worn. Some newer types of implants allow the artificial eye to move, but they're not yet able to provide vision.
Children who have an artificial eye can still participate in sports, as long as they use proper protective eyewear.
Cryotherapy (sometimes called cryosurgery), is used to treat small tumors or additional tumors that develop. It's often used together with chemotherapy, or it may be used after radiation therapy.
During cryotherapy, your child is first put to sleep by an anesthesiologist. Then the surgeon uses ultrasound to guide and place a small, extremely cold probe directly on the tumor. He or she is extremely careful to ensure that the surrounding healthy tissue remains unharmed. Cryotherapy may be repeated on several occasions, if necessary.
An advantage of this procedure is that it may help prevent the need for enucleation or radiation therapy. The major disadvantages are that it may leave a scar which damages vision, and it's not very effective in treating larger tumors.
Thermotherapy is a method of delivering heat to the eye using ultrasound, microwaves or infrared radiation. Like cryotherapy, it's only useful for very small tumors, and can be combined with chemotherapy or radiation therapy. Thermotherapy leaves a relatively small scar, and tends to preserve more vision than cryotherapy.
Photocoagulation, also called light coagulation, is another method for treating small tumors. This technique uses focused light from a laser to destroy the tumor by destroying its blood supply. It's sometimes used in combination with chemotherapy.
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy:
- External radiation therapy uses a machine outside the body to send radiation toward the cancer.
- Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, plaques or catheters that are placed directly into or near the cancer.
The way the radiation therapy is given depends on the type and stage of the cancer being treated. Methods of radiation therapy used to treat retinoblastoma include:
- Intensity-modulated radiation therapy (IMRT) —A type of 3-dimensional (3-D) radiation therapy that uses a computer to make pictures of the size and shape of the tumor. Thin beams of radiation of different intensities (strengths) are aimed at the tumor from many angles. This type of radiation therapy causes less damage to healthy tissue near the tumor.
- Stereotactic radiation therapy—Radiation therapy that uses a rigid head frame attached to the skull to aim high-dose radiation beams directly at the tumors, causing less damage to nearby healthy tissue. You may also here it called stereotactic external-beam radiation or stereotaxic radiation therapy.
- Proton beam radiation therapy—Radiation therapy that uses protons made by a special machine. A proton is a type of high-energy radiation that is different from an x-ray.
- Plaque radiotherapy—Radioactive seeds are attached to one side of a disk, called a plaque, and placed directly on the outside wall of the eye near the tumor. The side of the plaque with the seeds on it faces the eyeball, aiming radiation at the tumor. The plaque helps protect other nearby tissue from the radiation.
While surgery, radiation and chemotherapy can be very helpful in helping your child battle retinoblastoma, they may come with side effects:
- Radiation often produces inflammation, which can temporarily make symptoms and dysfunction worse.
- Some chemotherapy agents are associated with fatigue, diarrhea, constipation and headache. These side effects can usually be effectively managed with standard medical approaches.
Our cancer treatment center has specialists who deliver complementary or alternative medicines. These treatments, which may help control pain and side effects of therapy, include:
- therapeutic touch
- herbal supplements
- dietary recommendations
Talk to your child's physician about whether complementary or alternative medicine might be a good option for your child.
New types of treatment being tested in clinical trials
1. Subtenon chemotherapy
Subtenon chemotherapy uses drugs injected through the membrane covering the muscles and nerves at the back of the eyeball. This is a type of regional chemotherapy. It's usually combined with systemic chemotherapy and local treatment (such as radiation therapy, cryotherapy, photocoagulation or thermotherapy).
2. Ophthalmic arterial infusion therapy
Ophthalmic arterial infusion therapy is a type of regional chemotherapy used to deliver anticancer drugs directly to the eye. A catheter is put into an artery that leads to the eye and the drug is given through the catheter. During this treatment, a small balloon may be inserted into the artery to block it and keep most of the drug trapped near the tumor.
3. High-dose chemotherapy with stem cell transplant
High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.
4. Biologic therapy
Biologic therapy is a treatment that uses your child's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy. Clinical trials for retinoblastoma are studying a biologic therapy called gene therapy. This is a treatment that changes a gene to improve the body's ability to fight the disease.
Check for U.S. clinical trials for intraocular retinoblastoma from National Cancer Institute's PDQ Cancer Clinical Trials.
Check for U.S. clinical trials for extraocular retinoblastoma from National Cancer Institute's PDQ Cancer Clinical Trials.
What is the long-term outlook for children with retinoblastoma?
As with any cancer, the prognosis can vary greatly from child to child. Luckily, the survival rate is very high and prompt medical attention and aggressive therapy usually contribute to your child's best outcome.
What will long-term care for my child be like?
Lifelong follow-up care is crucial if your child has been treated for retinoblastoma—one reason is that he's more likely to develop a different type of cancer elsewhere in his body. The most common of these secondary cancers is osteosarcoma (cancer of the bone). Retinoblastoma has also been linked to melanoma, breast, lung, bladder and other types of cancers much later in life. Researchers are working to understand why these secondary cancers occur in survivors of retinoblastoma.
It's important for your child to visit a survivorship clinic every year to:
- manage disease complications
- screen for recurrence of cancer and/or secondary cancers
- manage late effects of treatment
A typical follow-up visit may include some or all of the following:
- physical exam
- laboratory testing
- imaging scans
Through the David B. Perini, Jr. Quality of Life Clinic at Dana-Farber Cancer Institute, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team.
Coping and support
In addition to providing medical care, we strive to also meet all of your family's emotional and quality-of-life needs. We know that unfamiliar places, especially hospitals, often create anxiety and fear for a child. We also know this isn't an easy time or task for parents, who have their own fears and concerns about the situation. Our family support services will help address many of your needs and concerns. Read about our resources and support services.