Retinoblastoma

The retina is the light-sensitive tissue at the back of the eyeball. Retinoblastoma is a cancer of the retina that happens mostly to young children.

• Retinoblastoma can run in families

• It usually happens before age 2

• About 1 in 4 children with retinoblastoma have it in both eyes

• A child with retinoblastoma may have a white pupil, crossed eyes, or vision problems

• Treatment includes surgery, chemotherapy, and sometimes radiation therapy 

Retinoblastoma is caused by a problem with the genes that control eye development. A baby can inherit this problem from a parent or the problem can happen on its own.

Symptoms include:

• A pupil (the black spot in the center of the eye) that's white instead of black

• Eyes that look in different directions (strabismus, also called cross-eyes)

• If the retinoblastoma is large, vision problems

If the cancer has spread, children may also throw up, lose their appetite, or have a headache.

Leukocoria in Infant With Retinoblastoma

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A white pupil (leukocoria) is a symptom of retinoblastoma.

By permission of the publisher. From Scott I, Warman R, Murray T: Atlas of Ophthalmology. Edited by RK Parrish II and TG Murray. Philadelphia, Current Medicine, 2000.

If a doctor suspects a retinoblastoma, they'll:

• Do a special eye exam while your child is asleep under general anesthesia

• Ultrasound, CT (computed tomography) scan, or MRI (magnetic resonance imaging) to see the retina

If your child has retinoblastoma, doctors will do more tests to see if the cancer has spread, including:

• Bone scan

• Taking a sample of bone marrow (bone marrow biopsy) to look for cancer

• Sometimes, a spinal tap

Doctors will also do genetic testing to see if your child's retinoblastoma is the type that can be passed down in a family. If it is, doctors will also test members of the child's family. Brothers and sisters at risk for retinoblastoma should have eye exams every 4 months from birth to age 4. Parents' eyes should also be checked, because the same gene can cause noncancerous tumors in an adult retina.

Treatment depends on where the retinoblastoma is and whether it has spread. It's important to remove the whole tumor, but if possible, doctors use treatments that spare the vision.

If retinoblastoma is only in one eye, doctors usually:

• Remove the eye

If the retinoblastoma is in both eyes, doctors may remove the eye with the largest tumor and try to treat the other eye without removing it so your child can still see. Those treatments may use:

• Chemotherapy drugs injected directly through the main artery that provides blood to the eye

• Radiation therapy

• Cryotherapy

• Laser therapy

• Pieces of radioactive material placed near the tumor (brachytherapy)

After treatment, specialists should examine your child regularly to make sure the cancer hasn't come back.