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Retinoblastoma - Childhood

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What is retinoblastoma?

Most cancers are named for the part of the body where the cancer starts. Retinoblastoma is a cancer that starts in the retina, the very back part of the eye. It is the most common type of eye cancer in children. Rarely, children can have other kinds of eye cancer, such as medulloepithelioma, which is described briefly below. But the information in this document focuses on retinoblastoma and not other kinds of eye cancer.

To understand retinoblastoma, it helps to know something about the normal structures of the eye and how they work.

About the eye

The main part of the eye is the eyeball (also known as the globe), which is filled with a jelly-like material called vitreous. The front of the eyeball has a lens with an iris (the colored part of the eye that acts like a camera shutter), which allows light to enter the eye and focuses it on the retina.

The retina is the inner layer of cells in the back of the eye. It is made up of specialized nerve cells that are sensitive to light. These light-sensing cells are connected to the brain by the optic nerve, which runs out the back of the eyeball. The pattern of light (image) that reaches the retina is sent through the optic nerve to an area of the brain called the visual cortex, allowing us to see.

How does retinoblastoma develop?

The eyes develop very early as babies grow in the womb. During the early stages of development, the eyes have cells called retinoblasts that divide into new cells and fill the retina. At a certain point, the cells stop dividing and develop into mature retinal cells.

Rarely, something goes wrong with this process. Instead of developing into special cells that detect light, some retinoblasts continue to grow rapidly and out of control, and form a cancer known as retinoblastoma.

The chain of events that lead to retinoblastoma is complex, but it starts with an abnormality (mutation or change) in a gene called the retinoblastoma (Rb or RB1) gene. The normal RB1 gene helps keep cells from growing out of control. Depending on when and where the change in the RB1 gene occurs, 2 different types of retinoblastoma can result.

Congenital (hereditary) retinoblastoma : In about 1 out of 3 retinoblastomas, the abnormality in the RB1 gene is congenital (present at birth) and is in all the cells of the body (known as a germline mutation). This includes all of the cells of both retinas.

In most of these children, there is no family history of this cancer. Only about 25% of the children born with this gene abnormality inherit it from a parent. In about 75% of cases the gene change first occurs during early development in the womb. The reasons for this are not clear.

Children born with a mutation in the RB1 gene usually develop retinoblastoma in both eyes (bilateral retinoblastoma). Within the eyes there are often several tumors (multifocal retinoblastoma).

Because all of the cells in the body have the changed RB1 gene, these children also have a higher risk of developing cancers elsewhere in the body.

A small number of children with this form of retinoblastoma will develop another tumor in the brain, usually in the pineal gland at the base of the brain (a pineoblastoma). This is also known as trilateral retinoblastoma.

For survivors of hereditary retinoblastoma, the risk of developing other cancers later in life is also higher than average. (For more information.

Sporadic (non-hereditary) retinoblastoma

In about 2 out of 3 cases of retinoblastoma, the abnormality in the RB1 gene develops on its own in only one cell in one eye. It is not known what causes this change. A child who has sporadic (non-hereditary) retinoblastoma develops only one tumor in one eye. This type of retinoblastoma is often found at a later age than the hereditary form.

How does retinoblastoma grow and spread?

If retinoblastoma tumors are not treated, they can continue to grow and may fill much of the eyeball. Cells may break away from the main tumor on the retina and float through the vitreous to reach other parts of the eye, where they can form more tumors. If these tumors block the channels that let fluid circulate within the eye, the pressure inside the eye can rise. This can cause glaucoma, a possible serious complication of retinoblastoma, which can lead to pain and loss of vision in the affected eye.

Most retinoblastomas are found and treated before they have spread outside the eyeball. But retinoblastoma cells can occasionally spread to other parts of the body. The cells sometimes grow along the optic nerve and reach the brain. Retinoblastoma cells can also grow through the covering layers of the eyeball and into the eye socket, eyelids, and nearby tissues. Once tissues outside the eyeball are affected, the cancer may then spread to lymph nodes (small bean-shaped collections of immune system cells) and to other organs such as the liver, bones, and bone marrow (the soft, inner part of many bones).

Medulloepithelioma :  Medulloepithelioma is another type of eye tumor. It is not a type of retinoblastoma, but it is mentioned here because it also usually occurs in young children. These tumors are very rare.

Most medulloepitheliomas are malignant (cancerous), but they rarely spread outside the eye. They usually cause eye pain and loss of vision.

The diagnosis is made when a doctor finds a tumor mass in the eye by using an ophthalmoscope (an instrument that helps doctors to look inside the eye). Like retinoblastoma, the diagnosis is usually made based on the appearance and location of the tumor inside the eye. A biopsy (removing cells from the tumor to be looked at under a microscope) to confirm the diagnosis is almost never done because it might harm the eye or risk spreading the cancer outside of the eye.

Treatment for medulloepithelioma is almost always surgery to remove the eye.

What are the risk factors for retinoblastoma?

A risk factor is anything that affects a person’s chance of getting a disease such as cancer. But risk factors don’t tell us everything. Different cancers have different risk factors.

Lifestyle-related risk factors such as body weight, physical activity, diet, and tobacco use play a major role in many adult cancers. But these factors usually take many years to influence cancer risk, and they are not thought to play much of a role in childhood cancers, including retinoblastomas.

Age :  Most children diagnosed with retinoblastoma are younger than 3 years old. Most congenital or hereditary retinoblastomas are found during the first year of life, while non-inherited retinoblastomas tend to be diagnosed in 1- and 2-year-olds. Retinoblastomas are rare in older children and in adults.

Heredity : About 1 out of 3 cases of retinoblastoma are caused by a mutation (change) in the RB1 gene that is present in all the cells of the child’s body. But of these cases, only about 1 in 4 is inherited from one of the child’s parents. In the rest, the gene mutation has not been inherited, but has occurred during early development in the womb. Children born with a mutation in the RB1 gene usually develop retinoblastoma in both eyes. Regardless of whether the mutated RB1gene was inherited from a parent or not, because these children have the mutated gene in all of their cells, they have a 1 in 2 chance of eventually passing it on to their children.

The remaining 2 out of 3 cases occur as a result of a random RB1 gene mutation that occurs only in one cell of one eye. These cancers are not inherited from a parent, and children who have them do not pass on a greatly increased risk of retinoblastoma to their children. Non-hereditary retinoblastomas always affect one eye only.

The way in which inherited gene changes make certain children likely to develop retinoblastoma is explained in the next section, “Do we know what causes retinoblastoma?”

Do we know what causes retinoblastoma?

Retinoblastoma is caused by mutations (changes) in certain genes. Over the past few decades, scientists have made great progress in learning how certain changes in a person’s DNA can cause cells of the retina to become cancerous. DNA is the chemical in each of our cells that makes up our genes – the instructions for how our cells function. We usually look like our parents because they are the source of our DNA. But DNA affects much more than just the way we look.

Some genes have instructions that control when our cells grow, divide into new cells, and die. Certain genes that help cells grow and divide are called oncogenes. Others that slow down cell division or cause cells to die at the right time are called tumor suppressor genes. Cancers can be caused by DNA changes that turn on oncogenes or turn off tumor suppressor genes.

The most important gene in retinoblastoma is the RB1 tumor suppressor gene. This gene makes a protein (pRb) that helps stop cells from growing too quickly. Each cell normally has 2 RB1 genes. As long as a retinal cell has at least one RB1 gene that works as it should, it will not form a retinoblastoma. But when both of the RB1 genes are mutated or missing, a cell can grow unchecked. This can lead to further gene changes, which in turn may cause cells to become cancerous.

Hereditary or bilateral retinoblastoma : About 1 out of 3 children with retinoblastoma have a germline mutation in one RB1 gene; that is, all the cells in the body have the defective RB1 gene. Most of these children (75%) have developed this mutation after conception while in the womb. The other 25% have inherited it from one of their parents.

About 90% of children who are born with this germline mutation of the RB1 gene develop retinoblastoma. This happens when the second RB1 gene is lost or mutated. In most cases the retinoblastoma is bilateral (in both eyes), but sometimes it is found early enough that it is still only in one eye.

These children have hereditary retinoblastoma. All bilateral retinoblastomas are considered hereditary, although not all hereditary retinoblastomas are bilateral (in both eyes) when they are found.

Every person has 2 RB1 genes but passes only 1 on to each of their children (the child gets the other RB1 gene from the other parent). The odds that a parent who had hereditary retinoblastoma will pass the mutated gene on to his or her child are 1 out of 2.

Most children with hereditary retinoblastoma don’t have an affected parent (the RB1 mutation occurs while in the womb). But these children will eventually be at risk of passing the disease on to their children. This is why we call this form of retinoblastoma “hereditary” (even though neither of the child’s parents may have been affected).

Non-hereditary (sporadic) retinoblastoma : The remaining 2 out of 3 children with retinoblastoma do not have the RB1gene mutation in all the cells of the body. Instead, the RB1 gene mutation happens early in life and first occurs only in one cell in one eye.

Whether the changes in the RB1 gene are hereditary or sporadic, what causes these changes is not known. They may result from random gene errors that sometimes occur when cells reproduce and divide. There are no known lifestyle-related or environmental causes of retinoblastomas, so it is important to remember that there is nothing these children or their parents could have done to prevent these cancers.

Can retinoblastoma be prevented?

With many cancers in adults, the risk of cancer can be reduced by avoiding certain risk factors, such as smoking or exposure to hazardous chemicals in the workplace. But there are no known avoidable risk factors for retinoblastoma. So there is nothing that a parent or child could have done to prevent this cancer. If your child does develop retinoblastoma, it is important to realize that you or your child did nothing to cause it.

Some cases of retinoblastoma are hereditary, so survivors should consider genetic counseling before having children to understand the risks of passing the gene change on to their children and perhaps to explore ways to avoid this. For example, an option some people might consider would be to use in vitro fertilization and implant only embryos that don’t have the gene change.

If a preventive option is not used, children born into a family with a history of retinoblastoma should be screened for this cancer starting shortly after birth because early detection of this cancer greatly improves the chance for successful treatment.

Can retinoblastoma be found early?

Retinoblastoma is a rare cancer, and there are no widely recommended screening tests to look for retinoblastoma in children without symptoms. Still, many retinoblastomas are found early, either by a child’s doctor or by parents or relatives.

During children's regular physical exams, doctors routinely check their eyes. Some of the things doctors look for include changes in how the eyes look (inside or outside), changes in how the eyes move, and changes in the child’s vision. Any of these might be a sign of retinoblastoma, although they are more often caused by something else.

In some cases, a parent or relative may notice that a young child's eye has an unusual appearance, prompting a visit to the doctor. It is important for parents to be aware of the possible signs and symptoms of retinoblastoma, which are described in the section, “How is retinoblastoma diagnosed?”, and to report anything unusual to the doctor as soon as possible. Most often the cause is something other than retinoblastoma, but it is important to have it checked so that the cause can be found and treated right away, if needed.

For children in families known to have an abnormal RB1 gene, or in families with a history of retinoblastoma who have not had genetic testing of the RB1 gene, doctors recommend regular eye exams during the first years of life to detect tumors at an early stage. These children often have an eye exam a few days after birth, again at about 6 weeks of age, then every few months until at least age 5. Since this genetic defect can now be found by a special blood test, most doctors advise that children with parents or siblings with a history of retinoblastoma have this genetic test done during the first weeks after birth. The results of the test then help define how often eye exams should be done.

Most cases of hereditary retinoblastoma develop and are diagnosed in infants only a few months old. Usually, if tumors develop in both eyes, it happens at the same time. But in some children, tumors develop in one eye first, then a few months or up to a year or more later in the other eye. So even if retinoblastoma is diagnosed in only one eye, these children still need regular exams of the other eye for several years after treatment.

If a child has retinoblastoma that is thought to be hereditary, most doctors also recommend magnetic resonance imaging (MRI) scans of the brain at regular intervals for up to 5 years to check for a trilateral retinoblastoma (a brain tumor such as a pineoblastoma). 

How is retinoblastoma diagnosed?

Most types of cancer can be found by physical exam and imaging tests, but treatment is usually not begun until the diagnosis is confirmed by a biopsy. During a biopsy, the doctor removes a sample from the tumor and sends it to a lab to be looked at under a microscope.

But biopsies are not usually done to diagnose retinoblastoma for 2 reasons. First, taking a biopsy specimen from a tumor in the eye cannot be done easily without harming the eye and risking spreading the cancer cells outside of the eye. Second, retinoblastoma can be diagnosed accurately by doctors who have experience with this disease, and it is unlikely to be confused with other eye problems in children.

Signs and symptoms

Retinoblastomas are often found when a parent or doctor notices a child's eye looks unusual.

White pupillary reflex: This is the most common early sign of retinoblastoma. Normally when you shine a light in a child's eye, the pupil (the dark spot in the center of the eye) looks red because of the blood in vessels in the back of the eye. In an eye with retinoblastoma, the pupil often appears white or pink instead, which is known as a white pupillary reflex. This white glare of the eye may be noticed by a parent after a flash photograph is taken, especially if the pupils are different colors. It may also be noted by the child's doctor during a routine eye exam.

Lazy eye: Sometimes both eyes do not appear to look in the same direction, a condition often called lazy eye. (Doctors call this strabismus.) There are many possible causes of this in children. Most of the time lazy eye is caused by a mild weakness of the muscles that control the eyes, but retinoblastoma is also one of the rare causes.

Less common symptoms and signs of retinoblastoma include:

Vision problems

Eye pain

Redness of the white part of the eye

Bleeding in the front part of the eye

Bulging of the eye

A pupil that does not get smaller when exposed to bright light

A different color in each iris (the colored part of the eye)

Medical history and physical exam

If your child has signs or symptoms that might be due to retinoblastoma, the doctor will want to examine your child's eyes and take a complete medical history. The doctor will probably ask about the family history of retinoblastoma or other cancers and about the child's symptoms. This information is important when deciding if more tests and exams by specialists are needed. Your family history is also useful for determining whether other relatives could possibly pass this gene on to their children or develop this cancer themselves (if they are young children) and might benefit from genetic counseling.

If a retinoblastoma is suspected, the doctor will refer you to an ophthalmologist (a doctor who specializes in eye diseases), who will examine the eye closely to be more certain about the diagnosis. The ophthalmologist will use special lights and magnifying lenses to look inside the eye. Usually, the child needs to be under general anesthesia (asleep) during the exam so that the doctor can take a careful and detailed look.

If a diagnosis of retinoblastoma seems likely, imaging tests will be done to help confirm it and to find out how far it may have spread within the eye and possibly to other parts of the body. Usually an ophthalmologist who specializes in treating cancers of the eye will make the final determination. This doctor should also be part of the team of doctors treating the cancer.

Imaging tests : Imaging tests use x-rays, sound waves, magnetic fields, or radioactive substances to create pictures of the inside of the body. The tests themselves are painless, but some may require injections. Imaging tests may be done for a number of reasons, including:

To help distinguish between retinoblastoma and other eye diseases

To determine how large the cancer is and how far it has spread

To help determine if treatment has been effective

Children with retinoblastoma may have one or more of these tests.

Ultrasound : Ultrasound, also known as ultrasonography, uses sound waves to create images of tissues inside the body, such as the inner parts of the eye. For this test, a small ultrasound probe is placed on the surface of the eye. The probe releases sound waves and detects the echoes that bounce off the tissues inside and around the eye. The echoes are converted by a computer into a black and white image of the eye and nearby tissues that is displayed on a computer screen.

Ultrasound is one of the most commonly used imaging tests to confirm the diagnosis of retinoblastoma. It is painless and does not expose the child to radiation, but the child may need to be sedated (made sleepy) so that the doctor can get a good look at the eye. This test can be very useful when tumors in the eye are so large they prevent doctors from seeing inside the whole eye because ultrasound can “see through” tissues.

Computed tomography (CT) scan : The CT scan is an x-ray test that produces detailed cross-sectional images of parts of the body. Instead of taking one picture, like a regular x-ray, a CT scanner takes many pictures as it rotates around your child while he or she lies on a table. A computer then combines these pictures into images of slices of the part of the body being studied. Unlike a regular x-ray, a CT scan creates detailed images of the soft tissues in the body, such as the eye and surrounding structures.

Before the scan, your child may receive an IV (intravenous) injection of a contrast dye that helps better outline structures in the body. The dye may cause some flushing (a feeling of warmth, especially in the face). Some people are allergic and get hives. Rarely, more serious reactions like trouble breathing or low blood pressure can occur. Be sure to tell the doctor if your child has any allergies or has ever had a reaction to any contrast material used for x-rays.

CT scans take longer than regular x-rays, but not as long as MRI scans. Your child will need to lie still on a table while they are being done. During the test, the table slides in and out of the scanner, a ring-shaped machine that completely surrounds the table. Your child may need to be sedated before the test to stay still and help make sure the pictures come out well. Many medical centers now use spiral CT (also known as helical CT), which completes the scan more quickly. It also gives more detailed pictures and uses lower doses of radiation during the test.

CT scans can help determine the size of a retinoblastoma tumor and how much it has spread within the eye and to tissues near the eye. Normally, either a CT or an MRI scan (see the next section) is needed to do this, but usually not both. Because CT scans give off radiation, which might raise a child’s risk for other cancers in the future, most doctors now prefer to use MRI.

Magnetic resonance imaging (MRI) scan :  Like CT scans, MRI scans provide detailed images of soft tissues in the body, such as the eye and surrounding structures. But MRI scans use radio waves and strong magnets instead of x-rays, so there is no radiation involved. The energy from the radio waves is absorbed by the body and then released in a pattern formed by the type of body tissue and by certain diseases. A computer translates the pattern into a very detailed image of parts of the body. A contrast material called gadolinium may be injected into a vein before the scan to better see details.

MRI scans may take up to an hour. Your child may have to lie inside a narrow tube, which is confining and can be upsetting. Newer, more open MRI machines can help with this, but the test still requires staying still for long periods of time. The machines also make buzzing and clicking noises that may be disturbing. Young children may be given medicine to help keep them calm or even asleep during the test.

MRI is often used to evaluate retinoblastomas because it provides very detailed images and does not use radiation. This test is especially good at looking at the brain and spinal cord. Most children with retinoblastoma will have at least one MRI scan to assess their tumor. For children with bilateral retinoblastomas, many doctors continue to do MRI scans of the brain for several years after treatment to screen for tumors of the pineal gland (sometimes called trilateral retinoblastoma).

Bone scan : A bone scan can help show if the retinoblastoma has spread to the skull and other bones. Most children with retinoblastoma do not need to have a bone scan. It is normally used only when there is a strong reason to think retinoblastoma may have spread beyond the eye.

For this test, a small amount of low-level radioactive material is injected into a vein (intravenously, or IV). The material settles in areas of damaged bone throughout the entire skeleton over the course of a couple of hours. Your child then lies on a table for about 30 minutes while a special camera detects the radioactivity and creates a picture of the skeleton. This may require sedation.

This test shows the entire skeleton at once. Areas of active bone changes appear as “hot spots” on the skeleton – that is, they attract the radioactivity. These areas may suggest the presence of cancer, but other bone diseases can also cause the same pattern. To help tell these conditions apart, other imaging tests such as plain x-rays or MRI scans, or even a bone biopsy might be needed.

Other tests : Some other types of tests are not commonly needed for retinoblastomas, but they may be helpful in some situations.

Biopsy : For most cancers, a biopsy (removing a tissue sample from the tumor and looking at it under a microscope) is needed to make a diagnosis. Trying to biopsy a tumor at the back of the eye can often damage the eye and may spread tumor cells, so this is almost never done to diagnose retinoblastoma. Instead, doctors make the diagnosis based on eye exams and on imaging tests such as those listed above. This is why it is very important that the diagnosis of retinoblastoma is made by experts.

Lumbar puncture (spinal tap) : Retinoblastomas may grow along the optic nerve, which connects the eye to the brain. If the cancer has spread to the surface of the brain, cancer cells can often be found in samples of cerebrospinal fluid (the fluid that surrounds the brain and spinal cord). Most children with retinoblastoma do not need to have a lumbar puncture. It is normally used only when there is a reason to think retinoblastoma may have spread into the brain.

For this test, the doctor first numbs an area in the lower part of the back over the spine. The child is typically given something so they will sleep and not move during the procedure. This can help ensure the spinal tap is done cleanly. A small, hollow needle is then placed between the bones of the spine to withdraw a small amount of the fluid. The fluid is then looked at under a microscope to check for cancer cells.

Bone marrow aspiration and biopsy : These 2 tests may be done to see if the cancer has spread to the bone marrow, the soft, inner part of certain bones. These tests are usually not needed unless the retinoblastoma has spread to tissues next to the eye and doctors suspect that the cancer may have also spread through the bloodstream to the bone marrow.

The tests are typically done at the same time. The samples are usually taken from the back of the pelvic (hip) bone, but in some cases they may be taken from the sternum (breastbone) or other bones.

In bone marrow aspiration, the skin over the hip and the surface of the bone may be numbed with a local anesthetic. This test can be painful, so the child will probably be given other medicines to reduce pain or even be asleep during the procedure. A thin, hollow needle is then inserted into the bone, and a syringe is used to suck out (aspirate) a small amount of liquid bone marrow.

A bone marrow biopsy is usually done just after the aspiration. A small piece of bone and marrow is removed with a slightly larger needle that is twisted as it is pushed down into the bone. Once the biopsy is done, pressure is applied to the site to help stop any bleeding.

The samples are then looked at under a microscope to see if tumor cells are present.

How is retinoblastoma staged?

The stage of cancer is a description of how far the cancer has spread. The outlook (prognosis) for people with cancer depends, to a large extent, on the cancer's stage. The stage of a cancer is one of the most important factors in choosing treatment.

Retinoblastoma is staged based on the results of eye exams, imaging tests, and any biopsies that were done. These tests were described in the previous section, “How is retinoblastoma diagnosed?”

A staging system is a standard way for your child's cancer care team to describe how far a cancer has spread. Doctors use staging systems to predict the outlook for saving the child's vision, as well as for survival and the likelihood that certain treatments will be effective.

Several detailed systems can be used to stage retinoblastoma. For practical purposes, when determining the best treatment options, doctors often divide retinoblastomas into 3 main groups:

Intraocular: it is still within the eye

Orbital: it has spread to the eye socket

Metastatic: it has spread to distant parts of the body

In the United States, most retinoblastomas are diagnosed before they have spread outside of the eye, so staging systems that apply only to intraocular retinoblastoma are used most often in this country. There are 2 staging systems for intraocular retinoblastomas. It is important to know that regardless of the stage, almost all children with intraocular retinoblastoma can be cured if they are properly treated.

The Reese-Ellsworth staging system : Some doctors may still use the Reese-Ellsworth staging system to classify retinoblastomas that have not spread beyond the eye. This system can help determine the likelihood of preserving vision while still effectively treating the tumor.

This system was developed in the 1960s, when most children were being treated with external beam radiation therapy (EBRT). Terms such as favorable, doubtful, and unfavorable used in this staging system refer to the likelihood that the cancer could be treated effectively while preserving the affected eye. These terms do not refer to the likelihood of the child's survival. Indeed, more than 9 in 10 children with intraocular retinoblastomas are cured. The major challenge is saving their sight.

To explain the groupings below, it will help to define a few terms. The optic disk is the end of the optic nerve where it is attached to the retina. Retinoblastomas are diagnosed by looking at the retina through an ophthalmoscope, so doctors cannot measure their size directly using a ruler. Instead they compare the size of the tumor with the size of the optic disk, which is usually about 1.5 millimeters (1/16 inch) across. For example, a tumor estimated to be 3 times the size of the disk (3 disk diameters or 3 DD) would be about 4.5 millimeters (3/16 inch) across.

The equator is an imaginary line that divides the front and back halves of the eyeball.

The Reese-Ellsworth staging system divides intraocular retinoblastoma into 5 groups. The higher the group number, from 1 to 5, the lower the chance of controlling the retinoblastoma or of saving the eye or any useful vision.

Group 1 (very favorable for saving [or preserving] the eye)

1A: one tumor, smaller than 4 disc diameters (DD), at or behind the equator

1B: multiple tumors smaller than 4 DD, all at or behind the equator

Group 2 (favorable for saving [or preserving] the eye)

2A: one tumor, 4 to 10 DD, at or behind the equator

2B: multiple tumors, 4 to 10 DD, all at or behind the equator

Group 3 (doubtful for saving [or preserving] the eye)

3A: any tumor in front of the equator

3B: one tumor, larger than 10 DD, behind the equator

Group 4 (unfavorable for saving [or preserving] the eye)

4A: multiple tumors, some larger than 10 DD

4B: any tumor extending anteriorly (toward the front of the eye) to the ora serrata (front edge of the retina)

Group 5 (very unfavorable for saving [or preserving] the eye)

5A: tumors involving more than half of the retina

5B: vitreous seeding (spread of tumors into the gelatinous material that fills the eye)

This staging system was first designed to determine how useful radiation therapy might be in a given patient, but it can still provide some useful information about which current treatments might be most effective. For example:

A group 1 retinoblastoma very likely can be controlled with treatments such as chemotherapy, photocoagulation, cryotherapy, thermotherapy, brachytherapy, or external beam radiation therapy while still preserving vision in the eye.

A group 4 or especially group 5 retinoblastoma is very unlikely to be controlled with chemotherapy or radiation therapy. Even if it were controlled, the vision in the eye would be very poor.

International Classification for Intraocular Retinoblastoma : The International Classification for Intraocular Retinoblastoma is a newer staging system, which takes into account what has been learned about the disease in recent decades. Most doctors now use this system. It divides intraocular retinoblastomas into 5 groups, labeled A through E, based on the chances that the eye can be saved using current treatment options.

Group A :  Small tumors (3 mm across or less) that are confined to the retina and are not near important structures such as the optic disk (where the optic nerve enters the retina) or the foveola (the center of vision).

Group B : All other tumors (either larger than 3 mm or small but close to the optic disk or foveola) that are still confined to the retina.

Group C : Well-defined tumors with small amounts of spread under the retina (subretinal seeding) or into the gelatinous material that fills the eye (vitreous seeding).

Group D : Large or poorly defined tumors with widespread vitreous or subretinal seeding. The retina may have become detached from the back of the eye.

Group E : The tumor is very large, extends near the front of the eye, is bleeding or causing glaucoma (high pressure inside the eye), or has other features that mean there is almost no chance the eye can be saved.

Other staging systems : Other staging systems that include both intraocular retinoblastomas and those that have spread beyond the eye may be used by some doctors. These may be especially useful in countries where these cancers are more likely to have spread by the time they are found. For example, the American Joint Commission on Cancer (AJCC) has developed a staging system that takes into account 3 key pieces of information:

T: the size of the main (primary) tumor and how far it has grown within and outside of the eye

N: whether or not the cancer has reached the lymph nodes (small, bean shaped collections of immune cells, to which cancers sometimes spread)

M: whether or not the cancer has spread (metastasized) to distant sites, such as the bone marrow, brain, skull, or long bones

This system can be used to describe the extent of retinoblastomas in detail, particularly for those cases when the disease has spread outside of the eye.

Be sure to ask your child's doctor which system is being used, and what it means in your child's case.

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