If you are looking for information on this site about retinoblastoma (RB), you probably had this disease as a kid and are a long term survivor or know someone with this disease.

What Is RB

…(it’s not rhythm and blues!)

Retinoblastoma is a malignant (cancerous) tumor of the retina. The retina is the thin nerve tissue lining the back of your eye that senses light and forms images. It is the most common eye cancer in childhood, but is overall a very rare tumor. The most common age at diagnosis is 2 years old, and 95 percent of children with RB are diagnosed by age 4.


…What Does It Mean For Me

Unlike other childhood cancers, retinoblastoma is a disease that can be inherited. However, it can also occur with no known cause. When it occurs without a family history, we are unaware of any risk factors. You can get RB in one or both eyes. Generally children who have it in both eyes have the inherited form, while those with it in only one eye probably developed the disease with no known cause. Parents with the hereditary form of RB have a 50 percent chance of having a child with RB. For this reason, children with a family history of RB are routinely screened for this disease.

What does that mean for you?

If you are a long term survivor of RB, you should see a genetic counselor if you decide to have a baby. This may be irrelevant right now (unless you are sexually active), but it is something to keep in the back of your mind.

Treatment For Retinoblastoma…

Treatment of RB depends on the extent of disease at the time of diagnosis and the potential to maintain vision. As with any cancer, we want the best therapy to get rid of the disease but to cause the least amount of damage to the healthy cells around it.

  • If you had the form of RB with no known cause, you may have had an enucleation (removal of the eye). If this is the case, you have a prosthesis in place and have no vision from that eye.
  • You may have also received radiation therapy to your affected eye if you were treated as a young child. While this is extremely effective, as you know, it has significant impact on your vision. For this reason, other treatment options are being studied to better maintain vision.
  • Along with this, if you received radiation therapy for RB, you are at increased risk for developing secondary cancers later in life – 50 percent of the time occurring at the radiation site.
  • If you had the hereditary form of RB and the tumors were small, you may have been treated with chemotherapy. The type of chemotherapy used can put you at an increased risk of developing a secondary leukemia. However this usually occurs within 3 to 5 years of your treatment, so at this point you are likely out of the woods.