Retinoblastoma
What is Retinoblastoma?
Retinoblastoma is a malignant (cancerous) tumor that occurs in the eye. Specifically, it arises in the part of the eye known as the retina, which is located along the back wall of the inside of the eye. Retinoblastoma is relatively rare occurring in 1 in 18,000 births and often is diagnosed in children who are less than two years of age. It more commonly occurs in only one eye. Individuals who have both eyes affected have approximately a 50% chance of having a child with retinoblastoma.
What are the symptoms of Retinoblastoma?
Retinoblastoma is hard to diagnose early because the symptoms are not obvious. You may have been the first to notice a white reflection in your child's pupil called the cat's eye reflex. This is when you can actually see the tumor as the eye moves and light reflects off the tumor. The child may have been squinting or having difficulty focusing both eyes on the same object. This is called strabismus and usually occurs if the tumor is located in or very near the visually most sensitive part of the eye. Symptoms occurring in later stages of the disease include a painful red eye and loss or decrease of vision in the affected eye.
How is Retinoblastoma diagnosed?
Retinoblastoma is easily confused with other less severe eye conditions and careful examination is needed for accurate diagnosis. Many times an ophthalmologist may help with the examination, which is done in the operating room so that the entire retina can be examined. This procedure will be done under general anesthesia where medication is given so that the child is not conscious and will not feel any pain.
What is the treatment for Retinoblastoma?
If the examination shows a great amount of disease in one eye, treatment may include removal of that eye. If both eyes are involved, the eye that is affected the most is removed and the other eye is treated. Removal of your child's eye is done only when the disease has progressed beyond hope of saving useful vision.
The treatment depends on the size and location of the tumor and whether the tumor has spread. Rarely, retinoblastoma can spread to places outside of the eye as well. The most common places are the bone marrow, the central nervous system and the bones. After diagnosis, a number of tests will be done to see if the tumor cells have spread. These tests may include a bone marrow aspirate and biopsy, spinal tap, CT scans and bone scans. Several types of treatment are available. These include surgery, radiation, chemotherapy, cryotherapy and laser therapy. Surgery and radiation are the most common treatments for retinoblastoma. Treatment is usually given over three to five weeks. If the tumor has not spread, retinoblastoma is treated with radiation.
Complications of radiation therapy can include slight damage to the blood vessels, which should not reduce vision. Extensive radiation can eventually lead to glaucoma, increased pressure in the eye. Radiation of the lens of the eye can result in cataracts, clouding of the lens of the eye. In advanced cases surgery to remove the effected eye is the usual treatment. Cryotherapy, or freezing of the tumor, may be used to treat small localized tumors.
When surgery or radiation treatment is finished, your child may be given chemotherapy to destroy any remaining traces of disease. Some of the chemotherapy medicines may be Cyclophosphamide, Vincristine, Actinomycin D, Adriamycin, VCR, VP-16 and Carboplatinum. These drugs have all been found to be somewhat effective against retinoblastoma and may be used alone or in combinations for a course of chemotherapy. After the treatment is completed, the child will receive periodic check-ups, including eye examinations under anesthesia and X-rays. If needed, your child can be fitted with a prosthesis or glass eye.
What is the prognosis for Retinoblastoma?
Success in treating retinoblastoma can be measured either by survival or preservation of vision. Generally, about 90% of children with retinoblastoma survive for five years or longer. However, most children with advanced disease have major changes in their vision as a result of the disease. The prognosis when the tumor has metastasized is not as favorable.
There is strong evidence that when retinoblastoma occurs in both eyes, there is a hereditary factor.
Treatment may be more successful due to early screening of children who have a family history of the disease.
Contact Us
For additional information on this topic, please contact us, cancer@cchmc.org.
Written 8/05
