Jagdish Kathwate
Retinoblastoma - Patient Education
What is retinoblastoma?
Retinoblastoma is a rare cancer of the retina, the thin membrane on the inside back of the eye that is stimulated by light. Retinoblastoma is usually diagnosed before a child reaches the age of 3.
Retinoblastoma can be hereditary (passed down in families) or non-hereditary.
• Forty percent of retinoblastoma patients have a genetic defect that leads to multiple tumors in one eye or both eyes. This is known as hereditary or germline retinoblastoma. These patients are typically diagnosed before 1 year of age.
• Patients with hereditary retinoblastoma may pass this disease to their children.
• Throughout their lives, patients with hereditary retinoblastoma are more likely to develop other cancers inside and outside of their eyes.
• Sixty percent of patients have the nonhereditary form of retinoblastoma. Each of these patients develops a tumor in only one eye. Nonhereditary patients are diagnosed on average around 2 years of age.

Untreated, retinoblastoma can spread widely:
• throughout the retina
• throughout the fluid inside the eye (also called the vitreous). Large tumors may detach from the retina and break into smaller tumors, called vitreous seeds. Floating in the vitreous, these seeds are very difficult to treat.
• Into the tissue under the retina
• into the eye socket, optic nerve and brain
• to the bones and the bone marrow

How common is retinoblastoma?
• Retinoblastoma accounts for about 3 percent of all childhood cancers.

What are the symptoms of retinoblastoma?
Symptoms of retinoblastoma may include:
• “Cat’s eye”: a white-yellow mass or glow seen through the pupil—often first noticed in a photo of a child’s face when the flash is used without “red eye reduction.” Normally, the center of the eye appears red in response to the camera flash, but in retinoblastoma, the center of the eye may have a white glow.
• Complaints of poor vision
• One or both eyes turning inward or outward
• Pain from increased pressure in the eye as the tumor grows

How is retinoblastoma treated?
Treatment of retinoblastoma depends on whether:
• the tumor affects one eye or both eyes
• the center of vision is affected by the tumor
• the tumor has spread to other parts of the body
Treatment can include surgery, chemotherapy, focal therapy and radiation therapy.
• Surgery— is used to remove the eye, if needed.
• Eye(s) may be removed in children with advanced retinoblastoma.
• In patients with only one affected eye: When that one eye is removed, more than 90 percent of those patients do not need any more treatment.
• In patients with both eyes affected: If one eye is removed, treatment will focus on saving the remaining eye.
When the tumor has spread into the tissues surrounding the eye or the eye socket, the patient is treated with chemotherapy after surgery.
• Chemotherapy (“chemo”)— uses powerful medicines to kill cancer cells or stop them from growing (dividing) and making more cancer cells:
• Chemo may be injected into the blood stream, so that it can travel throughout the body.
• Chemo may also be injected around the eye (periocular) for local treatment.
• Combination therapy uses more than one type of chemo at a time.
Chemotherapy alone cannot cure retinoblastoma, so patients often receive “focal therapy.”
• Focal therapy—is treatment with laser therapy or freezing treatments (cryotherapy) while under anesthesia (medicine to help the child sleep). These focal therapies may continue even after chemotherapy is complete. Sometimes, the tumors in the eye are small enough to be treated with only focal therapy.
Very rarely the tumor invades the brain or spreads to the bones or bone marrow. These patients require more intensive chemo, usually including a stem cell transplant.
• Stem cell transplant— includes replacing blood-forming cells in the bone marrow that have been killed by chemo and/or radiation therapy:
• a stem cell transplant gives the patient new immature blood cells from a donor’s blood or bone marrow. These cells grow into healthy blood cells to replace the ones the patient lost.
• Some types of stem cell transplants may be called “bone marrow transplants” because the cells come from the donor’s bone marrow.
Sometimes only radiation therapy, the strongest treatment for fighting retinoblastoma, helps maintain vision and prevent the spread of the cancer.
• Radiation therapy— uses high-energy X-rays or other types of radiation to kill cancer cells or stop them from growing. Radiation is reserved for patients who have not responded to other therapies.
• External radiation uses machines outside the body to deliver the X-ray dose.
• Internal radiation uses needles, seeds, wires or catheters (tubes) to deliver the radiation directly into or close to the cancer.
A very young child with multiple tumors or retinoblastoma in both eyes is likely to keep developing retinoblastoma tumors until 3 years of age. So, treatment might be less aggressive until age 3:
• Chemotherapy— to shrink the tumors
• Focal therapy— laser therapy and certain freezing methods done under anesthesia

What are the survival rates for retinoblastoma?
• If the tumor is contained within the eye (one or both eyes), more than 95 percent of treated patients can be cured.
• For children with retinoblastoma in both eyes, about 70 to 80 percent of the eyes can be saved.

Retinoblastoma Retinoblastoma 01/03/2011
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