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This information is produced and provided by the National Cancer Institute (NCI). The information in this topic may have changed since it was written. For the most current information, contact the National Cancer Institute via the Internet web site at http://cancer.gov or call 1-800-4-CANCER.
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:
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:
High birth weight and larger than expected size at birth are linked with an increased risk of embryonal rhabdomyosarcoma.
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:
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:
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
For patients with recurrent cancer, prognosis and treatment depend on the following:
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 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:
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:
The staging system is based on the size of the tumor, where it is in the body, and whether it has spread to other 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 said to be an "unfavorable" site.

Pea, peanut, walnut, and lime show tumor sizes.
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:
Stage 4
In stage 4, the tumor may be any size and cancer may have spread to nearby lymph nodes . Cancer has also spread to distant parts of the body such as the lung , bone marrow, or bone.
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 found only in the place where it started and it was completely removed by surgery . Tissue was taken from the edges of where the tumor was removed. The tissue was checked under a microscope by a pathologist and no cancer cells were seen.
Group II
Group II is divided into groups IIA, IIB, and IIC.
Group III
Cancer was partly removed by surgery and there are cancer cells (a lump or mass ) remaining that can be seen by x-ray or other imaging test . Cancer has 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 risk group is based on the staging system and the grouping system and is used to plan treatment.
The risk group describes the chance that rhabdomyosarcoma will recur (come back). The following risk groups are used:
Low-risk childhood rhabdomyosarcoma
Low-risk childhood rhabdomyosarcoma is one of the following:
Intermediate-risk childhood rhabdomyosarcoma
Intermediate-risk childhood rhabdomyosarcoma is one of the following:
High-risk childhood rhabdomyosarcoma
High-risk childhood rhabdomyosarcoma may be the embryonal type or the alveolar type. It may have spread to nearby lymph nodes and has spread to one or more distant parts of the body.
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.
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 health care providers who are experts in treating cancer in children.
Because rhabdomyosarcoma can form in many different parts of the body, many different kinds of treatments are used. Treatment will be overseen by a pediatric oncologist , a doctor who specializes in treating children with cancer. The pediatric oncologist works with other health care providers who are experts in treating children with rhabdomyosarcoma and who specialize in certain areas of medicine . These may include the following specialists:
Some cancer treatments cause side effects months or years after treatment has ended.
Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment for rhabdomyosarcoma may include:
Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.)
Three types of standard treatment are used:
Surgery
Surgery (removing the cancer in an operation) is used to treat childhood rhabdomyosarcoma. A type of surgery called wide local excision is often done. A wide local excision is the removal of tumor and some of the normal tissue around it, including the 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:
For most children with rhabdomyosarcoma, complete removal of the tumor by surgery is not possible.
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, and sometimes radiation therapy, may be given before surgery to shrink large tumors.
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 lower the risk that the cancer will come back, 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.
Types of external radiation therapy include the following:
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 cerebrospinal fluid , 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.
See Drugs Approved for Rhabdomyosarcoma for more information.
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.
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.
Immunotherapy
Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biologic therapy or biotherapy.
Targeted therapy
Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells.
Monoclonal antibody therapy is a type of targeted therapy being studied in the treatment of childhood rhabdomyosarcoma. Monoclonal antibody therapy uses antibodies made in the laboratory, from a single type of immune system cell. These antibodies can identify substances on cancer cells or normal substances that may help cancer cells grow. The antibodies attach to the substances and kill the cancer cells, block their growth, or keep them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry drugs, toxins, or radioactive material directly to cancer cells. Monoclonal antibodies may be used in combination with chemotherapy as adjuvant therapy.
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 listing of clinical trials.
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.
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
Rhabdomyosarcoma of the arms or legs
Rhabdomyosarcoma of the chest or abdomen
Rhabdomyosarcoma of the area near the testicles
Rhabdomyosarcoma of the bladder and prostate
Rhabdomyosarcoma of the vagina, vulva, or uterus
Rhabdomyosarcoma in unusual areas
Metastatic rhabdomyosarcoma
Chemotherapy options
Every child treated for rhabdomyosarcoma should receive chemotherapy . The dose of the chemotherapy and the number of treatments given depend on the child's risk group, as follows:
Low-risk patients
Intermediate-risk patients
High-risk patients
Radiation therapy options
Radiation therapy may be used if childhood rhabdomyosarcoma tumor cells remain after surgery , after chemotherapy , or if the tumor is of the alveolar type.
Treatment may include certain kinds of radiation therapy that cause less damage to normal tissue and lessen late effects of treatment. These include:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with previously untreated childhood rhabdomyosarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Recurrent Childhood Rhabdomyosarcoma
Treatment options for recurrent childhood rhabdomyosarcoma are based on many factors, including where in the body the cancer has come back, what type of treatment the patient had before, and the needs of the individual child. Treatment may include one or more of the following:
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent childhood rhabdomyosarcoma. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
For more information from the National Cancer Institute about childhood rhabdomyosarcoma, see the following:
For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Changes were made to this summary to match those made to the health professional version.
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PDQ is a comprehensive cancer database available on NCI's Web site.
PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.
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The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.
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PDQ also contains information on clinical trials.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness.
Listings of clinical trials are included in PDQ and are available online at NCI's Web site. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
The PDQ database contains listings of groups specializing in clinical trials.
The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
Last Revised: 2012-02-03
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