What is Retinoblastoma?

What is Retinoblastoma?
The newer stuff is at the bottom of the page!

I state here, on my page, that I'm not a doctor. I want to make sure everyone knows that. I am, as stated elsewhere, in the medical field, and I've done some research on the subject of Retinoblastoma. Here's my explanation of what Retinoblastoma is. For the most, up-to-date and late breaking information, try one of my LINKS.

Retinoblastoma (RB) is a term which refers to the genetic condition causing tumors to grow on the Retina of the eye. Unilateral RB simply means that tumors have grown in only one eye. Unilateral might not be caused by genetic, but rather a spontaneous growth. Bilateral RB is when a tumor or tumors have grown in both eyes and usually indicates a genetic influence (either spontaneous mutation or inherited).

If you break Retino-blast-oma into it's separate components you can begin to get an idea of what it's about.

Retino refers to the Retina which is a part of our eye (see diagram below).
Blast is a medical term used to indicate that the tumor is derived from the "primitive" cell. Any tumor name with "blast" in it indicates that it's from the "infantile" cell, and almost always occur in children, before their cells mature.
Lastly "oma", which just means "tumor" or "mass".

The Retina is the part of the eye that converts light that enters the eye to be converted to chemical and ultimately electrical impulses that are sent to the brain for interpretation. Light enters the eye through the lens and is "projected" on the retina. If this path is interrupted, sight is also interrupted.

As I understand it, all of us who do not have RB, have a gene on the 13th chromosome, which basically tells our body that we're not suppose to have any tumor cells growing on our retina that are different from the usual retina cells. People who have RB, do not have this gene. Since there is nothing telling their eye not to have those "other" cells, they grow out of control, thus creating "tumors". It is these dreaded "tumors" that we have to fight on a regular basis.

When a child is born with bilateral RB, but does not have anyone in their family who has the disease, they are said to have had a genetic "mutation". There are "environmental" factors all around us that could cause these "mutations". It's said to be part of evolution. Most mutations are good and allow us to adapt to our environment. This particular mutation, in my opinion is not particularly good (even though a side effect of RB seems to have a high IQ associated with it). Environmental factors can include: "background radiation" (naturally occurring radiation from the sun or naturally occurring radioactive rocks) from natural sources or sometimes from medical exams. Some other environmental factors can be from such things as chemicals in the water we drink, pollution in the air we breathe. It could be as simple as "old age".

Now that medical knowledge is getting better, more of these children are able to grow up to have children of their own. When a person with RB has a child, they have a good chance of passing on their genes to their child, increasing the risk of the child developing RB also.

Treatment for RB has come a long way. Thirty-something years ago, when my wife Lisa was being treated for RB, she was given external beam radiation, and chemo. Her check-ups were in a office with several people just holding her down so she wouldn't move. In fact; the only reason the tumor in the eye that she still has was seen, was because they had put her under general anesthesia to remove the first eye. Now, EUA's (Examination Under Anesthesia) are common. In our our son's case, it wasn't necessary for Chemo, or External beam radiation. Instead, we had several Laser treatments, Cryotherapy (freezing treatments), and the use of Radioactive plaques (a gold shield with radioactive material that delivers radiation directly to the tissues affected by the tumor). Most of the time, the Laser and Cryotherapy were performed while he was having his EUA.

It would appear that most of the research now is in the field of genetic studies. If they can figure out how to replace that "missing" gene, then the body would be able to fight the tumors on it's own. This would reduce the number of tumors and, at the same time, save sight.

I'm not a doctor, but I am in the medical field. Here's a little diagram which might explain some of the things that you might hear your doctor talking about.

a - Sclera
a1 - Cornea
b - Choroid
d - Iris
e - Retina
g - Optic Disc
h - Retinal Arteries
i - Vitreous body
j - Aqueous Humor
k - Lens
n - Conjunctiva
o - Optic Nerve

Today I was reading through some new information about RB HERE and came across some interesting information.

As of yet, I have not heard from anyone else who has had a tumor directly in the Macula. The Macula is the part of the eye that we use to read with. The Macula being different from the rest of the eye because there is a larger concentration of certain cells which allows for a clearer sight "area".

Well.... This study showed that the location of the eye appears to have a direct correlation with the child's age. It says in short that the younger the child is when they are diagnosed (thus when the tumor first began to grow) the tumor grows closer to the center or Macula. And, conversely the older the child the further from the center the tumors would grow.

It also mentions that most tumors discovered in the Macula are diagnosed at or near birth. In fact the incidence of finding a tumor in the Macula after the age of 15.5 months. I thought this was pretty interesting and maybe you might find it interesting as well.

The conclusion (copied directly) reads

CONCLUSION: There is a time course in which tumors in different parts of the retina come to clinical presentation. All macular tumors in this study and the majority of tumors in the posterior pole were detected before age 24 months. The authors provide possible explanations for these findings and their implications for treatment.

What is Tri-lateral RB? Tri-lateral Retinoblastoma is caused from the same thing as Bi-lateral. That is a gene that doesn't work properly. Everyone who does not have Bilateral or Trilateral have a gene that says "when a tumor starts growing on or in this sort tissue, KILL IT" RB people this gene doesn't work, so they get tumors. Once the cells in the retina "mature" they don't get tumors on them like when they are still "Inmature". So where do the Tri-lateral come in? There is a gland in the center of the brain called the pineal gland. The cells that make up the pineal gland, if looked under a microscope, look just like the cells that make up the retina. So..... you can get tumors growing there too. So after RB is considered a threat, Tri-lateral also is no longer a threat. It's actually a little younger. For RB, most people stop having EUA's now at age 3 or 2 years after their last active tumor. Tri-lateral cases usually show up within the first year and a half. Bilateral is rare enough, Tri-lateral happens in only a small percentage of those, so it's rarity is even greater. And.. Like Cathy Helms put it.... very few survive (if any). Copied directly from This page is the "long" explanation! Good luck (HEHE)
"Trilateral retinoblastoma" (TRB) is the association of a midline intracranial tumor with familial bilateral retinoblastoma. Classical- ly, the intracranial tumor is a pineal region tumor (pineoblastoma) with histology similar to retinoblastoma. We present a 7-month-old child with bilateral orbital retinoblastoma with a third tumor arising in the chiasmatic cistern. The presentation and prognosis of patients with this association differ from those children with "classical" TRB. The presentation, treatment, and outcome are described, with a review of the literature.

I welcome any suggestions. If this helps you, please let me know. Also if you find anything that you know to be inaccurate or false, I would appreciate being enlightened.
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