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Table of Contents General Information About Childhood Rhabdomyosarcoma
Stages of Childhood Rhabdomyosarcoma
Recurrent Childhood Rhabdomyosarcoma
Treatment Option Overview
Treatment Options for Childhood Rhabdomyosarcoma
To Learn More About Childhood Rhabdomyosarcoma
Get More Information From NCI
Changes to This Summary (07/01/2008)
About PDQ
General Information About Childhood Rhabdomyosarcoma
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Childhood rhabdomyosarcoma is a disease in which malignant (cancer) cells form in muscle tissue.
Rhabdomyosarcoma is a type of sarcoma. Sarcoma is cancer of soft tissue (such as muscle), connective tissue (such as tendon or cartilage), and bone. Rhabdomyosarcoma usually begins in muscles that are attached to bones and that help the body move. Rhabdomyosarcoma is the most common type of sarcoma found in the soft tissues of children. It can occur in many places in the body.
There are three main types of rhabdomyosarcoma:
- Embryonal: This type occurs most often in the head and neck area or in the genital or urinary organs. It is the most common type.
- Alveolar: This type occurs most often in the arms or legs, chest, abdomen, or genital or anal areas. It usually occurs during the teen years.
- Anaplastic: This type rarely occurs in children.
See the following PDQ treatment summaries for more information about sarcomas:
Certain genetic conditions increase the risk of childhood rhabdomyosarcoma.
Anything that increases the risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. Parents who think their child may be at risk should discuss this with the child's doctor. Risk factors for rhabdomyosarcoma include having the following inherited diseases:
In most cases, the cause of rhabdomyosarcoma is not known.
A possible sign of childhood rhabdomyosarcoma is a lump or swelling that keeps getting bigger.
Lumps and other symptoms may be caused by childhood rhabdomyosarcoma. The symptoms that occur depend on where the cancer forms. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:
- A lump or swelling that keeps getting bigger or does not go away. It may be painful.
- Bulging of the eye.
- Headache.
- Trouble urinating or having bowel movements.
- Blood in the urine.
- Bleeding in the nose, throat, vagina, or rectum.
Tests that examine the area of the body with symptoms are used to detect (find) and diagnose childhood rhabdomyosarcoma.
The following tests and procedures may be used:
- Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
- X-ray: An x-ray of the organs and bones inside the body. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the abdomen or pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
- MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
- Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.
- Lumbar puncture: A procedure used to collect cerebrospinal fluid (CSF) from the spinal column to check for cancer cells. This is done by placing a needle into the spinal column. This procedure is also called an LP or spinal tap.
- Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
- Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. The biopsy is done after imaging tests are done. If rhabdomyosarcoma is found, the pathologist will determine the type. Because treatment depends on the type of rhabdomyosarcoma, patients should ask to have biopsy samples checked by a pathologist who has experience in diagnosing rhabdomyosarcoma.
- Bone marrow aspiration and biopsy: The removal of bone marrow, blood, and a small piece of bone by inserting a hollow needle into the hipbone. Samples are removed from both hipbones. A pathologist views the bone marrow, blood, and bone under a microscope to look for signs of cancer.
- Light and electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.
- Immunohistochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of cancer tissue to test for certain antigens. This type of study is used to tell the difference between different types of cancer.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
- Where in the body the tumor started.
- The size of the tumor at the time of diagnosis.
- Whether the tumor can be completely removed by surgery.
- Whether the tumor has spread to lymph nodes or distant parts of the body.
- The type of rhabdomyosarcoma.
- The patient's age and general health.
- Whether the tumor has just been diagnosed or has recurred (come back).
Stages of Childhood Rhabdomyosarcoma
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After childhood rhabdomyosarcoma has been diagnosed, treatment is based on the stage of the cancer and whether cancer remains after surgery to remove the tumor.
The process used to find out if cancer has spread within the muscle or to other
parts of the body is called staging. It is important to know the stage in order to plan treatment. The doctor will use results of the diagnostic tests 4 to help determine the stage of the disease.
Treatment for childhood rhabdomyosarcoma is based on the stage and the amount of cancer that remains after surgery to remove the tumor. The pathologist will use a microscope to check the tissues, including lymph nodes, removed during surgery, and the edges of the areas where the cancer was removed. This is done to see if all the cancer cells were taken out during the surgery.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
- Through tissue. Cancer invades the surrounding normal tissue.
- Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
Staging of childhood rhabdomyosarcoma is done in three related steps.
Childhood rhabdomyosarcoma is staged by using three different ways to describe the cancer:
- A grouping system.
- A staging system.
- A risk group.
The grouping system is based on whether the cancer has spread and how much cancer remains after surgery to remove the tumor:
Group I
Cancer was only in the place where it started and it was completely removed by surgery. No cancer cells are seen when tissue taken from the edges of where the tumor was removed is viewed under a microscope by a pathologist.
Group II
Group II is divided into groups IIA, IIB, and IIC.
- IIA: Cancer was removed by surgery but there are cancer cells remaining that can be seen only with a microscope.
- IIB: Cancer had spread to nearby lymph nodes and the cancer and lymph nodes were removed by surgery.
- IIC: Cancer had spread to nearby lymph nodes and the cancer and lymph nodes were removed by surgery. There are cancer cells remaining that can be seen only with a microscope.
Group III
Cancer was removed by surgery and there are cancer cells remaining that can be seen without a microscope. Cancer did not spread to distant parts of the body.
Group IV
Cancer had spread to distant parts of the body at the time of diagnosis.
The staging system is based on tumor location and size, and whether it has spread to lymph nodes or distant parts of the body:
Stage 1
In stage 1, cancer is any size, has not spread to lymph nodes, and is found in only one of the following "favorable" sites:
Rhabdomyosarcoma that occurs in a "favorable" site has a better prognosis. If the site where cancer occurs is not one of the "favorable" sites listed above, it is considered an "unfavorable" site.
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Stage 2
In stage 2, cancer is found in any one area not included in stage 1. The tumor is 5 centimeters or smaller and has not spread to lymph nodes.
Stage 3
In stage 3, cancer is found in any one area not included in stage 1 and one of the following is true:
- The tumor is 5 centimeters or smaller and cancer has spread to nearby lymph nodes.
- The tumor is larger than 5 centimeters and cancer may have spread to nearby lymph nodes.
Stage 4
In stage 4, the tumor may be any size and cancer may have spread to nearby lymph nodes. Cancer has spread to distant parts of the body such as the lung, bone marrow, or bone.
The risk group is based on both the grouping system and the staging system and is used to plan treatment.
The following risk groups are used:
Low-risk childhood rhabdomyosarcoma
Low-risk childhood rhabdomyosarcoma is one of the following:
- An embryonal tumor of any size that is found in a "favorable" site. There may be tumor remaining after surgery that can be seen without a microscope. The cancer may have spread to lymph nodes. The following areas are "favorable" sites:
- An embryonal tumor of any size that is not found in one of the "favorable" sites listed above. There may be tumor remaining after surgery that can be seen only with a microscope. The cancer may have spread to lymph nodes.
Intermediate-risk childhood rhabdomyosarcoma
Intermediate-risk childhood rhabdomyosarcoma is one of the following:
- An embryonal tumor of any size that is not found in one of the "favorable" sites listed above. There is tumor remaining after surgery, that can be seen with or without a microscope. The cancer may have spread to nearby lymph nodes.
- An alveolar tumor of any size in a "favorable" or "unfavorable" site. There may be tumor remaining after surgery that can be seen with or without a microscope. The cancer may have spread to nearby lymph nodes.
High-risk childhood rhabdomyosarcoma
High-risk childhood rhabdomyosarcoma may be the embryonal type, alveolar type, or anaplastic type. It may have spread to nearby lymph nodes and has spread to distant parts of the body.
Recurrent Childhood Rhabdomyosarcoma
Recurrent childhood rhabdomyosarcoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the same place or in other parts of the body. Treatment Option Overview
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There are different types of treatment for patients with childhood rhabdomyosarcoma.
Different types of treatments are available for children with rhabdomyosarcoma. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
Children with rhabdomyosarcoma should have their treatment planned by a team of doctors with expertise in treating cancer in children.
Because rhabdomyosarcoma can form in many different parts of the body, many different kinds of treatments are used. Your child's care will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care professionals who are experts in treating children. These may include the following specialists:
Some cancer treatments cause side effects months or years after treatment has ended.
Some cancer treatments cause side effects that continue or appear months or years after cancer treatment has ended. These are called late effects. Late effects of cancer treatment for rhabdomyosarcoma may include:
- Physical problems.
- Changes in mood, feelings, thinking, learning, or memory.
- Second cancers (new types of cancer).
Some late effects may be treated or controlled. It is important to talk with your child's doctors about the possible late effects caused by some treatments. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer 5 for more information.)
Three types of standard treatment are used:
Surgery
Surgery (removing the cancer in an operation) is used to treat childhood rhabdomyosarcoma. Surgery is usually wide local excision, which is removal of the tumor and some of the normal tissue around it, including lymph nodes. When an extra amount of normal tissue is removed from around the tumor, it is called an en bloc removal of a cuff of normal tissue. A second surgery may be needed to remove all the cancer. Whether surgery is done and the type of surgery done depends on the following:
- Where in the body the tumor started.
- The effect the surgery will have on the child's appearance.
- The effect the surgery will have on the child's important body functions.
- How the tumor responded to chemotherapy or radiation therapy that may have been given first.
Rhabdomyosarcoma can form in many different places in the body and the surgery will be different for each site. Surgery to treat rhabdomyosarcoma of the eye or genital areas is usually a biopsy. Chemotherapy or radiation therapy may be given before surgery to shrink large tumors.
For most children with rhabdomyosarcoma, complete removal of the tumor by surgery is not possible. Even if the doctor removes all the cancer that can be seen at the time of the surgery, patients will be given chemotherapy, with or without radiation therapy, after surgery to kill any cancer cells that are left. Treatment given after the surgery, to increase the chances of a cure, is called adjuvant therapy.
Radiation therapy
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or stop them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated. The type and amount of radiation therapy and when it is given depend on where in the body the tumor started, how much tumor remained after surgery, and the age of the child.
Chemotherapy
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is treatment using more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web
site 6.
High-dose chemotherapy with stem cell transplant
High-dose chemotherapy with stem cell transplant is a way of giving high doses of chemotherapy and replacing blood-forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's clinical trials database.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Childhood Rhabdomyosarcoma
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.
Previously Untreated Childhood Rhabdomyosarcoma
Surgery options
Childhood rhabdomyosarcoma may be treated with chemotherapy and/or radiation therapy before or after surgery. Treatment with surgery may include the following:
Rhabdomyosarcoma of the head and neck
- For tumors of the head and neck that are close to the skin and not in or near the eye: Treatment may include surgery (wide local excision). Some lymph nodes may be removed from the same side of the neck as the tumor. Surgery will be followed by chemotherapy with or without radiation therapy.
- For tumors of the head and neck that are in or near the eye: A biopsy of the tumor is followed by chemotherapy and radiation therapy. If the tumor remains or comes back after treatment with chemotherapy and radiation therapy, surgery to remove the eye and some tissues around the eye may be needed.
- For tumors of the head and neck that cannot be removed by surgery: Chemotherapy and radiation therapy are given.
Rhabdomyosarcoma of the arms or legs
- For tumors of the arms or legs: Surgery (wide local excision and en bloc removal of a cuff of normal tissue) may be done. For tumors in the arms, lymph nodes near the tumor and in the armpit area may be removed. For tumors in the legs, lymph nodes near the tumor and in the groin area may be removed. A second surgery may be done to remove any remaining tumor cells.
Rhabdomyosarcoma of the chest or abdomen
- For tumors in the chest wall or abdominal wall: Surgery (wide local excision) may be done. A second surgery may be done to remove any remaining tumor cells.
- For tumors of the chest or abdomen: Surgery alone or chemotherapy and radiation therapy, followed by surgery to remove the tumor.
- For large tumors of the chest or abdomen: Chemotherapy and/or radiation therapy is given to shrink the tumor first, followed by surgery (wide local excision) to remove the remaining tumor.
- For tumors of the muscles or tissues around the anus or between the vulva and the anus or the scrotum and the anus: Surgery is done to remove as much of the tumor as possible and some nearby lymph nodes, followed by chemotherapy.
- For tumors of the gallbladder or bile ducts: Surgery is done to remove as much of the tumor as possible, followed by chemotherapy.
Rhabdomyosarcoma of the area near the testicles
- Rhabdomyosarcoma of the testicular area is usually treated with surgery to remove the testicle and spermatic cord. Sometimes the lymph nodes in the back of the abdomen are removed, especially if the child is older than 9 years. CT scans may be done every 3 months after surgery to see if the cancer is growing in nearby lymph nodes.
Rhabdomyosarcoma of the prostate or bladder
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