Childhood Cancer: Retinoblastoma
Written by Dr. Abby Trettin
What is Retinoblastoma?
Retinoblastoma is a rare type of malignant cancer that usually develops in early childhood, typically before age 5. This form of cancer develops in the retina, the thin layer of nerve cells in the back of the eye that is responsible for detecting light and color. It occurs when nerve cells in the retina change, growing in size and number and eventually forming a tumor. The cells usually spread in and around the eye and can also spread to other parts of the body, including the brain and spine. This advanced form of retinoblastoma can be life threatening. In about two-thirds of cases, only one eye is involved.
How Common is Retinoblastoma?
Retinoblastoma is diagnosed in 250 to 350 children per year in the United States. This equates to about 1 in 14,000-18,000 live births. It accounts for approximately 4 percent of all cancers in children younger than 15 years of age.
What are the Signs and Symptoms of Retinoblastoma?
- Leukocoria – visible whiteness in the pupil
- Particularly noticeable in dim light or with flash photography
- Strabismus – crossed eyes
- Change in color of iris
- Redness, soreness, or swelling of eyelids
- Eye appears to protrude or bulge forward
- Blindness or poor vision in affected eye
How is Retinoblastoma Diagnosed?
Any signs of leukocoria require an emergent visit to your optometrist. The doctor will thoroughly examine the child for signs of retinoblastoma. The diagnosis is made based upon a thorough clinical evaluation, detailed patient history, identification of characteristic symptoms, and the use of a variety of specialized tests. The child will need to be dilated in order to complete an examination of the interior of the eye. If the doctor is unable to get a thorough look at the back of the eye, the child may need to be referred for an examination under anesthesia. This will allow for proper identification of the tumor. An MRI may be ordered to determine the extent of the tumor and to determine if the tumor has spread to surrounding tissues. CT scans are generally avoided because of the potential risk of additional radiation-induced tumors if the child has hereditary retinoblastoma.
How is Retinoblastoma Treated?
Treatment is directed first toward preserving life, then towards preserving vision. The treatment has to be highly personalized based on the individual. The available options depend on the tumor and whether it has spread to other areas of the eye or body. Most treatments require the coordinated efforts of a team of specialists, which can include: optometrists, pediatricians, surgeons, pediatric ophthalmologists, pediatric oncologists, radiation oncologists, ocularists, and social workers.
Specific treatment methods depend on several factors, including: size of tumor, exact location of tumor, extend of primary tumor, degree of malignancy, the individual’s age and general health, and the associated likelihood of retaining adequate vision. The following are the current treatment options for retinoblastoma:
- Chemotherapy – drug based therapy that targets cancer cells to shrink the tumor
- Radiation Therapy – uses a beam of energy to kill cancer cells
- In some cases of retinoblastoma, a small disk of radioactive material is placed in or near the tumor and left in place for some time
- Laser Therapy – a laser is used to destroy blood vessels that feed the tumor, killing the cancer cells
- Cryotherapy (cold treatments) – an extremely cold substance, such as liquid nitrogen, is used to freeze cancer cells. Once the cells freeze, the substance is removed and the cells thaw out. Repeated freezing and thawing kills cancer cells
- Thermotherapy (heat treatments) – Extreme heat is targeted at the cancer cells
- Surgery (enucleation) –
- If the tumor has grown too much for other treatments, surgery may be needed to remove the eyeball. This may keep the cancer from spreading to other areas of the body
- Thankfully with advancements in treatment options, the rate of enucleation has dropped significantly in the past decades
- In those children that do undergo this procedure, the cure rate is approximately 90%
The 5-year survival rate for children with retinoblastoma is 95%.
Please schedule your child for a comprehensive eye exam immediately if you notice any of the signs or symptoms of retinoblastoma. If you have any questions or concerns, please feel free to call Dr. Hoppe or Dr. Trettin.
Boyd, K. 2019. What is Retinoblastoma? American Academy of Ophthalmology. https://www.aao.org/eye-health/diseases/what-is-retinoblastoma
Hurwitz, R. 2017. Retinoblastoma. Rare Disease Database. https://rarediseases.org/rare-diseases/retinoblastoma/
Retinoblastoma. 2017. Genetics Home Reference. https://ghr.nlm.nih.gov/condition/retinoblastoma#
Retinoblastoma – Childhood: Statistics. 2019. Cancer.net. https://www.cancer.net/cancer-types/retinoblastoma-childhood/statistics