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rhabdomyosarcoma-childhood

Rhabdomyosarcoma - Childhood

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What is rhabdomyosarcoma?

Sarcomas are cancers that develop from connective tissues in the body, such as muscles, fat, bones, membranes that line the joints, or blood vessels. There are many types of sarcomas. Rhabdomyosarcoma (RMS) is a cancer made up of cells that normally develop into skeletal muscles. The body has 3 main types of muscles.

Skeletal (voluntary) muscles are muscles that we control to move parts of our body.

Smooth muscle is the main type of muscle in internal organs (except for the heart). For example, smooth muscles in the intestines push food along as it is digested. We do not control this movement.

Cardiac muscle is the main muscle type in the heart.

About 7 weeks into the development of an embryo, cells called rhabdomyoblasts (which will eventually form skeletal muscles) begin to form. These are the cells that can develop into rhabdomyosarcoma. Because this is a cancer of embryonal cells, it is much more common in children, although it does sometimes occur in adults.

We think of our skeletal muscles as being mainly in our arms and legs, but these skeletal muscle cancers can start nearly anywhere in the body. Common sites include:

Head and neck (near the eye, inside the nasal sinuses or throat, or near the spine in the neck)

Urinary and reproductive organs (bladder, prostate gland, or any of the female organs)

Arms and legs

Trunk (chest and abdomen)

Rhabdomyosarcomas can even start in some parts of the body that don't normally have skeletal muscle.

Types of rhabdomyosarcoma

There are 2 main types of rhabdomyosarcomas, along with some less common types.

Embryonal rhabdomyosarcoma : Embryonal rhabdomyosarcoma (ERMS) is the most common type of rhabdomyosarcoma. It usually affects infants and young children. The cells of ERMS look like the developing muscle cells of a 6- to 8-week-old embryo. ERMS tends to occur in the head and neck area, bladder, vagina, or in or around the prostate and testicles.

Two subtypes of ERMS, botryoid and spindle cell rhabdomyosarcomas, tend to have a better prognosis (outlook) than the more common form of ERMS.

Alveolar rhabdomyosarcoma : Alveolar rhabdomyosarcoma (ARMS) typically affects older children or teens and occurs more often in large muscles of the trunk, arms, and legs. ARMS cells look like the normal muscle cells seen in a 10-week-old fetus. ARMS tends to grow faster than ERMS and usually requires more intensive treatment.

Anaplastic rhabdomyosarcoma and undifferentiated sarcoma : Anaplastic rhabdomyosarcoma (formerly called pleomorphic rhabdomyosarcoma) is an uncommon type that occurs in adults but is very rare in children.

Some doctors also group undifferentiated sarcomas with the rhabdomyosarcomas. Doctors can tell that these cancers are sarcomas using lab tests, but the cells don't have any features that help classify them further.

Both of these uncommon cancers tend to grow quickly and usually require intensive treatment.

Rhabdomyosarcoma in adults : Most rhabdomyosarcomas occur in children, but they can also occur in adults. Adults are more likely to have faster-growing types of rhabdomyosarcoma and to have them in parts of the body that are harder to treat. Because of this, rhabdomyosarcomas in adults are often harder to treat effectively.

This document focuses on rhabdomyosarcomas in children, but most of the information here (including much of the treatment information) applies to rhabdomyosarcomas in adults as well.

What are the risk factors for rhabdomyosarcoma?

A risk factor is anything that affects the chance of having a disease such as cancer. Different cancers have different risk factors. For example, smoking is a risk factor for several types of cancer in adults.

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 rhabdomyosarcoma (RMS).

No environmental factors (such as exposures during the mother's pregnancy or in early childhood) are known to increase the chance of getting RMS.

Age and gender : RMS is most common in children younger than 10, but it can also occur in teens and adults. It is slightly more common in boys than in girls.

Inherited conditions : Some people inherit a tendency to develop certain types of cancer. The DNA we inherit from our parents may have certain changes that account for this tendency. Some rare inherited conditions increase the risk of RMS (and usually some other tumors as well).

Members of families with Li-Fraumeni syndrome are more likely to develop sarcomas (including RMS), breast cancer, leukemia, and some other cancers.

Children with Beckwith-Wiedemann syndrome have a high risk of developing Wilms tumor, a type of kidney cancer, but they may also develop RMS.

Neurofibromatosis type 1, also known as von Recklinghausen disease, usually causes multiple nerve tumors (especially in nerves of the skin), but it also increases the risk of RMS.

Costello syndrome is a very rare congenital abnormality. Children with this syndrome have high birth weights but then fail to grow well and are short. They also tend to have a large head. They are prone to develop RMS as well as other tumors.

Noonan syndrome is a condition in which children are short, have heart defects, and are slower than typical children in developing physical skills and learning things. They are also at higher risk for RMS.

These conditions are rare and account for only a small fraction of RMS cases. But they suggest that the key to understanding RMS will come from studying genes and how they work in very early life to control cell growth and development.

Do we know what causes rhabdomyosarcoma?

We still do not know what causes most cases of rhabdomyosarcoma (RMS), but researchers are starting to understand how certain changes in DNA can cause normal cells to become cancerous. DNA is the chemical in each of our cells that makes up our genes – the instructions for how our cells function. It is packaged in chromosomes (long strands of DNA in each cell). We normally have 23 pairs of chromosomes in each cell (one set of chromosomes comes from each parent). We usually look like our parents because they are the source of our DNA. But DNA affects more than how we look.

Some genes are instructions for controlling 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.

For example, people with Li-Fraumeni syndrome have changes in the TP53 tumor suppressor gene that cause it to make a defective p53 protein. The p53 protein normally causes cells with DNA damage to either pause and repair that damage or, if repair is not possible, to self-destruct. When p53 is not working, cells with DNA damage continue to divide, causing further defects in other genes that control cell growth and development. This may lead to cancer.

Certain genes in a cell can be activated when bits of DNA are switched from one chromosome to another. This type of change, called a translocation, can happen when a cell is dividing into 2 new cells. This seems to be the cause of most cases of alveolar rhabdomyosarcoma (ARMS). In these cancers, a small piece of chromosome 2 (or, less often, chromosome 1) ends up on chromosome 13. This moves a gene called PAX3 (or PAX7 if it's chromosome 1) right next to a gene called FOXO1. The PAX genes play an important role in causing cells to grow while an embryo's muscle tissue is being formed, but these genes usually shut down once they're no longer needed. The normal function of the FOXO1 gene is to activate other genes. Moving them together likely activates the PAX genes, which may be what leads to the tumor forming.

Research suggests that embryonal rhabdomyosarcoma (ERMS) develops in a different way. Cells of this tumor have lost a small piece of chromosome 11 that came from the mother, and it has been replaced by a second copy of that part of the chromosome from the father. This seems to cause the IGF2 gene on chromosome 11 to be overactive. TheIGF2 gene codes for a protein that may cause these tumor cells to grow.

Changes in several different genes are usually needed for normal cells to become cancer cells. Scientists have found other gene changes that set some RMS cells apart from normal cells, but there are likely others that have not yet been found.

Researchers now understand many of the gene changes that may lead to RMS, but it's still not clear what might cause these changes. Some gene changes may be inherited. Others may just be a random event that sometimes happens inside a cell, without having an external cause. There are no known lifestyle-related or environmental causes of RMS, so it is important to know that there is nothing these children or their parents could have done to prevent these cancers.

Can rhabdomyosarcoma be prevented?

The risk of many adult cancers can be reduced with certain lifestyle changes (such as staying at a healthy weight or quitting smoking), but at this time there are no known ways to prevent most cancers in children.

The only known risk factors for rhabdomyosarcoma (RMS) – age, gender, and certain inherited conditions – cannot be changed. There are no known lifestyle-related or environmental causes of RMS, so at this time there is no way to protect against these cancers.

Even though we do not know how to prevent RMS, most children with this disease can be treated successfully.

Can rhabdomyosarcoma be found early?

At this time, there are no widely recommended screening tests for rhabdomyosarcoma (RMS). (Screening is testing for cancer in people who don't have any symptoms.)

Still, some cases of RMS can be found at an early stage because they start in parts of the body where they are noticed quickly for a list of common symptoms). For example, small tumors that start in the muscles behind the eye often cause the eye to bulge out. Tumors in the nasal cavity often cause nasal congestion, nosebleeds, or bloody mucus. When small lumps form near the surface of the body, parents often see them or feel them.

Many cases of RMS start in the bladder or other parts of the urinary tract and may cause trouble emptying the bladder or lead to blood in the urine or in diapers. Tumors starting around the testicles in young boys can cause painless swelling that is often noticed early by a parent. In girls with RMS of the vagina, the tumor may cause bleeding or a mucus-like discharge from the vagina.

It may be harder to recognize tumors in the arms, legs, and trunks of older children because they may often have pain or bumps from sports or play injuries.

There are many other causes of the symptoms above, and most of them are not serious, but it is important to have them checked by a doctor. This includes having your child's doctor check out any pain, swelling, or lumps that grow quickly or don't go away after a few weeks.

About 1 in 3 of these cancers is found early enough so that all of the visible cancer can be completely removed by surgery. But even when this happens, small microscopic tumors (which cannot be seen, felt, or detected by imaging tests) may have already spread to other parts of the body.

Families known to carry inherited conditions that raise the risk of this cancer (listed in "What are the risk factors for rhabdomyosarcoma?") or that have several family members with cancer (particularly childhood cancers) should talk with their doctors about the possible need for more frequent checkups. It is not common for this type of cancer to run in families, but close attention to possible early signs of cancer may help find it early, when treatment is most likely to be successful.

How is rhabdomyosarcoma diagnosed?

Certain signs and symptoms might suggest that a person may have rhabdomyosarcoma (RMS), but tests are typically needed to confirm the diagnosis.

Signs and symptoms of rhabdomyosarcoma

The symptoms of RMS depend largely on where the tumor is:

When the tumor is in the trunk, limbs, or groin (including the testicles), the first sign is usually a mass or swelling. Most often it doesn't cause any pain or other problems, but it may in some cases.

Tumors around the eye can cause the eye to bulge or the child to appear to be cross-eyed.

Tumors in the ear or nasal sinuses can cause an earache, headache, or sinus congestion.

Tumors in the bladder or prostate may lead to blood in the urine, while a tumor in the vagina may lead to vaginal bleeding. Any of these tumors may grow big enough to make it hard or painful to urinate or have bowel movements.

Tumors in the abdomen or pelvis can cause vomiting, abdominal pain, or constipation.

RMS rarely develops in the bile ducts (small tubes leading from the liver to the intestines), but when it does it can cause yellowing of the eyes or skin.

One or more of these symptoms usually leads parents to bring a child to the doctor. Less often, the child may have symptoms related to more advanced RMS, such as bone pain, constant cough, weakness, or weight loss.

Keep in mind that many of these signs and symptoms are more likely to be caused by something other than RMS. Still, if your child has any of these symptoms, check with your doctor so that the cause can be found and treated, if needed.

Medical history and physical exam : If your child has any signs or symptoms that may suggest RMS, the doctor will want to get a complete medical history to learn about any symptoms and how long your child has had them. The doctor will also examine your child to look for possible signs of RMS or other health problems. For example, the doctor may be able to see or feel an abnormal mass in the body.

If symptoms or the results of the physical exam suggest your child might have RMS, other tests will need to be done. These might include imaging tests, biopsies, and/or lab tests.

Imaging tests : Imaging tests use x-rays, magnetic fields, radioactive substances, or sound waves to create pictures of the inside of the body. Imaging tests may be done for a number of reasons, including:

To help find out whether a suspicious area might be cancerous

To determine the extent of a tumor or learn how far a cancer may have spread

To help determine if treatment is working

Patients who have or may have RMS will get one or more of these tests.

Plain x-rays : These are sometimes used to look for tumors, but their use is limited mainly to looking at bones because they do not show much detail in internal organs. An x-ray of the chest is sometimes done to look for cancer that might have spread to the lungs, although it isn't needed if a chest CT scan is being done.

Computed tomography (CT or CAT) scan : The CT scan is an x-ray test that produces detailed cross-sectional images of parts of the body, including soft tissues such as muscles. 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.

This test can provide fairly detailed information about a tumor, including how large it is and if it has invaded nearby structures. It can also be used to look at nearby lymph nodes, as well as the lungs or other areas of the body where the cancer might have spread.

Before the scan, your child may be asked to drink a contrast solution and/or get an intravenous (IV) injection of a contrast dye that helps better outline abnormal areas in the body. Your child may need an IV line through which the contrast dye is injected. The contrast 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. During the test, the table slides in and out of the scanner, a ring-shaped machine that completely surrounds the table. Your child will need to lie still on a table while this is being done. Younger children may be given medicine to help keep them calm or even asleep during the test. Many medical centers now use spiral CT (also known as helical CT), which completes the scan more quickly. It also yields more detailed pictures and lowers the dose of radiation received during the test.

Magnetic resonance imaging (MRI) scan :  Like CT scans, MRI scans give detailed images of soft tissues in the body. But MRI scans use radio waves and strong magnets instead of x-rays. The energy from the radio waves is absorbed 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 show details. The contrast material usually does not cause allergic reactions.

This test may be used instead of a CT scan to look at the tumor and the tissues around it. MRI is especially useful if the tumor is in certain parts of the body, such as the head and neck, an arm or leg, or the pelvis. MRI scans can help determine the exact extent of a tumor, as they provide a detailed view of the muscle, fat, and connective tissue around the tumor. This is important when planning surgery or radiation therapy. MRI is also very useful if your child's doctor is concerned about possible spread to the spinal cord or brain.

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

Bone scan : A bone scan can help show if a cancer has spread to the bones, and is often part of the workup for children with RMS. This test is useful because it provides a picture of the entire skeleton at once.

For this test, a small amount of low-level radioactive material is injected into a vein (IV). The substance 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. Younger children may be given medicine to help keep them calm or even asleep during the test.

Areas of active bone changes attract the radioactivity and show up as "hot spots" on the scan. These areas may suggest cancer in an area, but other bone diseases can also cause the same pattern, so other imaging tests such as plain x-rays or MRI scans, or even a bone biopsy might be needed.

Positron emission tomography (PET) scan : For a PET scan, a radioactive substance (usually a type of sugar related to glucose, known as FDG) is injected into the blood. The amount of radioactivity used is very low. Because cancer cells in the body are growing quickly, they absorb large amounts of the radioactive sugar.

After about an hour, your child will lie on a table in the PET scanner for about 30 minutes while a special camera creates a picture of areas of radioactivity in the body. The picture is not finely detailed like a CT or MRI scan, but it provides helpful information about the whole body.

PET scans are not used routinely to help diagnose RMS, but they can sometimes be very helpful in finding out if suspicious areas seen on other imaging tests (such as bone scans or CT scans) are tumors. PET scans can also be repeated during treatment to monitor the cancer over time.

Some newer machines are able to do a PET and CT scan at the same time (PET/CT scan). This allows the doctor to compare areas of higher radioactivity on the PET scan with the more detailed appearance of that area on the CT.

Ultrasound : Ultrasound uses sound waves and their echoes to make a picture of internal organs or tumors. For this test, a small, microphone-like instrument called a transducer is moved around on the skin (which is first lubricated with gel). It gives off sound waves and picks up the echoes as they bounce off the organs. The echoes are converted by a computer into an image that is displayed on a computer screen.

Ultrasound can be used to see if tumors in the pelvis (such as prostate or bladder tumors) are growing or shrinking over time. (This test can't be used to look at tumors in the chest because the ribs block the sound waves.)

This is an easy test to have done, and it uses no radiation. Your child simply lies on a table, and a technician moves the transducer over the part of the body being looked at.

Biopsy methods : The results of imaging tests may strongly suggest that someone has RMS, but a biopsy (removing some of the tumor for viewing under a microscope and other lab testing) is the only way to be certain. Usually several different kinds of lab tests are done on the sample to sort out what kind of tumor it is.

Biopsies can be done in several ways. The approach that is used will depend on where the mass is located, the age of the patient, and the expertise and experience of the doctor doing the biopsy.

Surgical biopsy : The most common biopsy approach is to surgically remove a small piece of tumor while the patient is under general anesthesia (asleep) and have it looked at by a pathologist (a doctor who diagnoses diseases from the results of lab tests). In some cases, nearby lymph nodes may also be removed and tested to see if the tumor has spread.

Needle biopsies: If for some reason a surgical biopsy cannot be done, a less invasive biopsy using a hollow needle may be used. There are 2 kinds of needle biopsies, each of which has pros and cons.

Core needle biopsy: For a core needle biopsy, the doctor inserts a hollow needle into the tumor to withdraw a piece of tissue (core sample). If the tumor is near the surface of the body, the doctor can guide the needle into the tumor by touch. But if the tumor is deep within the body, imaging tests such as ultrasound or CT scans may be needed to guide the needle into place. The core sample that is removed is then used in lab tests to help make the diagnosis.

The main advantage of a core needle biopsy is that it does not require surgery, so there is no large incision. Depending on where the tumor is, adults and older children may not need general anesthesia (where they are asleep for the biopsy), but some children may still need it. On the other hand, the specimen is smaller than with a surgical biopsy, and if it is not aimed correctly, the needle may miss the cancer. If the specimen is not a good sample of the tumor, another biopsy will be needed.

Fine needle aspiration (FNA) biopsy: This technique uses a very small hollow needle attached to a syringe to withdraw (aspirate) a small tumor sample. An FNA biopsy is ideally suited to tumors near the surface of the body that can be reached easily.

The downside of FNA is that the sample is very, very small. The pathologist must be experienced with this technique and be able to decide which lab tests will be most helpful on a very small sample. In cancer centers that have the experience to extract the most information from very small amounts of tissue, FNA can be a valuable – though certainly not foolproof – diagnostic approach, but it is not usually the preferred biopsy technique.

Bone marrow aspiration and biopsy : 

These tests aren't used to diagnose RMS, but they may be done after the diagnosis to find out if the tumor has spread to the bone marrow. The 2 tests are usually done at the same time. The samples are usually taken from the back of both of the pelvic (hip) bones, but in some patients they may be taken from the sternum (breastbone) or other bones.

These tests may be done during the surgery to treat the main tumor (while the child is still under anesthesia), or they may be done as a separate procedure.

If the bone marrow aspiration is being done as a separate procedure, the child lies on a table (on his or her side or belly). After cleaning the skin over the hip, the doctor numbs the area and the surface of the bone with local anesthetic, which may cause a brief stinging or burning sensation. Even with the local anesthetic, most patients still have some brief pain when the marrow is removed. In most cases, the child is also 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 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. The biopsy may also cause some brief pain. Once the biopsy is done, pressure will be applied to the site to help stop any bleeding.

Lumbar puncture (spinal tap) : Lumbar puncture is not a common test for RMS, but it may be done for tumors in the head near the covering of the brain (the meninges). This test is used to look for cancer cells in the cerebrospinal fluid (CSF), which is the liquid that bathes the brain and spinal cord.

For this test, the doctor first numbs an area in the lower part of the back near the spine. The doctor may also recommend that the child be given something to make him or her sleep so the spinal tap can be done without difficulty or causing harm. A small, hollow needle is then placed between the bones of the spine to withdraw some of the fluid.

Lab tests on the biopsy samples : A pathologist will look at the biopsy samples under a microscope to try to determine if they contain cancer cells. If the pathologist finds cancer, the next step is to figure out if the cancer is RMS. In rare cases, the pathologist can see that the cancer cells have small muscle striations (myofibrils), which confirm that the cancer is RMS. But in most cases, other lab tests are needed to confirm the diagnosis.

Pathologists may use special stains on the samples to identify the type of tumor. The stains contain special proteins (antibodies) that specifically attach to substances in RMS cells but not other cancers. The stains produce a distinct color that can be seen under a microscope. This lets the pathologist know that the tumor is a rhabdomyosarcoma.

Sometimes the tumor will also be tested for gene abnormalities. Genetic tests look for chromosome translocations and other DNA changes such as those discussed in the section "Do we know what causes rhabdomyosarcoma?"

If a diagnosis of RMS is made, the pathologist will also use these tests to help determine which kind of RMS your child has. This is important because it affects how the child is treated. For example, alveolar rhabdomyosarcoma (ARMS), which tends to be more aggressive, typically requires more intensive treatment than embryonal rhabdomyosarcoma (ERMS).

How is rhabdomyosarcoma staged?

Once the type of rhabdomyosarcoma (RMS) has been identified, doctors need to assess, as accurately as possible, how much of it there is and where it has spread. The answers to "how much" and "where" are expressed in a kind of shorthand known as staging.

The prognosis (outlook) 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.

Your child's doctors will use the results of the imaging tests and biopsies (described in "How is rhabdomyosarcoma diagnosed?") and the direct examination of the organs during surgery to try to determine how far the cancer has spread. If there is any doubt about the extent of the cancer, more biopsies may be done on tissues at the edge of the tumor, nearby lymph nodes, and any suspicious lumps in other parts of the body.

Staging for RMS is fairly complex. Doctors first determine 3 key pieces of information:

The type of RMS (embryonal or alveolar)

The TNM stage

The clinical group

These factors are then used along with the child's age to divide patients into risk groups, which then are used to determine the best treatment options.

The TNM stage

The TNM stage is based on 3 key pieces of information prior to treatment:

T: The characteristics of the tumor (location and size)

N: Whether the cancer has spread to nearby lymph nodes

M: Whether it has metastasized (spread) to distant parts of the body

These factors are combined to determine an overall stage:

Stage 1

The tumor started in a favorable area:

The orbit (area near the eye)

The head and neck area, except for parameningeal sites (next to the membranes covering the brain)

A genital or urinary site, except the bladder or prostate

Bile ducts (tubes leading from the liver to the intestines)

The tumor can be any size. It may have grown into nearby areas and/or spread to nearby lymph nodes, but it has not spread to distant sites.

Stage 2

The tumor started in an unfavorable site:

The bladder or prostate

An arm or leg

A parameningeal site (next to the membranes covering the brain)

Any other site not mentioned in stage 1

The tumor is 5 cm (about 2 inches) or smaller across and there is no evidence that it has spread to nearby lymph nodes or distant sites.

Stage 3

The tumor started in an unfavorable site:

The bladder or prostate

An arm or leg

A parameningeal site (next to the membranes covering the brain)

Any other site not mentioned in stage 1

And one of the following applies:

The tumor is 5 cm across or smaller but has spread to nearby lymph nodes

The tumor is larger than 5 cm across and may or may not have spread to nearby lymph nodes

In either case, the cancer has not spread to distant sites.

Stage 4

The tumor can have started at any site and can be of any size. It has spread to distant sites such as the lungs, liver, bones, or bone marrow.

Clinical group

The clinical group is based on the extent of the disease and how completely it is removed during initial surgery. The groups are defined as follows.

Group I

This group includes children with localized RMS (the cancer has not spread to nearby lymph nodes or to distant sites in the body) that is completely removed by surgery. Group I has 2 subgroups:

Group IA: Children in this group had a tumor that was still confined to the muscle or organ where it started and was completely removed by surgery. It had not spread to nearby lymph nodes or distant sites.

Group IB: Children in this group had a tumor that had grown beyond the muscle or organ where it started and into nearby structures, but it was completely removed by surgery. It had not spread to nearby lymph nodes or distant sites.

About 15% of RMS patients are in group I.

Group II

This group includes children who have had tumors that have been removed by surgery, but cancer has been found around the edges of the removed specimen, in the nearby lymph nodes, or in both places. In all cases, as much of the cancer has been removed as possible. Group II has 3 subgroups:

Group IIA: In this group, the cancer has not spread to nearby lymph nodes or elsewhere. The surgeon has removed all the cancer that could be seen, but the pathologist has found cancer at the edge of the removed specimen, which means that there may have been a small amount of cancer left behind.

Group IIB: In this group, the cancer has spread to nearby lymph nodes, but all of the cancer has been removed by surgery.

Group IIC: In this group, the cancer has spread to nearby lymph nodes. The surgeon has removed all the cancer that could be seen (including in the lymph nodes), but the pathologist has found cancer at the edge of the removed specimen, which means that there may have been a small amount of cancer left behind.

About 20% of RMS patients are in group II.

Group III

These children have tumors that cannot be completely removed, leaving some tumor behind that can be seen with the naked eye. The tumor may have spread to nearby lymph nodes, but there is no sign that it has spread to distant organs. Group III has 2 subgroups:

Group IIIA: The tumor cannot be completely removed by surgery, and only a biopsy of the tumor has been done.

Group IIIB: The tumor cannot be completely removed, but surgery has removed at least half of the tumor.

About 50% of patients with RMS are in group III.

Group IV

These children have evidence of distant spread at the time of diagnosis to places such as the lungs, liver, bones, bone marrow, or to distant muscles or lymph nodes.

About 15% of children with RMS are in group IV.

Risk groups

Using the information about the type of RMS, the TNM stage, and the clinical group, doctors classify patients into 3 risk groups. Information about risk groups helps doctors decide how aggressive treatment should be.

The risk groups are defined based on what has been learned from previous research on patients' outcomes. The risk groups discussed here are based on the most current information, but these may change in the future as safer and more effective treatments are developed.

Low-risk group

This group includes:

Children with TNM stage 1 embryonal rhabdomyosarcomas (ERMS) that fall into clinical groups I, II, or III

Children with stage 2 or 3 ERMS who are in clinical groups I or II

Intermediate-risk group

This group includes:

Children with stage 2 or 3 ERMS who are in clinical group III

Children with alveolar rhabdomyosarcoma (ARMS) that has not spread to distant sites (stage 1, 2, or 3)

High-risk group

This group includes:

Children with widespread (stage 4) RMS (ERMS or ARMS)

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