Childhood Rhabdomyosarcoma

Description

Note: The health professional version of this summary was extensively revised in June 2007. The patient version of this summary will reflect these changes shortly.

What is rhabdomyosarcoma?

Rhabdomyosarcoma is a disease in which cancer (malignant) cells begin growing in muscle tissue. Rhabdomyosarcoma is a type of a sarcoma, which means a cancer of the bone, soft tissues, or connective tissue (e.g., tendon or cartilage). Rhabdomyosarcoma begins in the soft tissues in a type of muscle called striated muscle. It can occur anywhere in the body.

Several types of sarcoma are found in children and young adults. The cancer cells must be looked at under a microscope to tell which type of sarcoma it is. Rhabdomyosarcoma is the most common type of sarcoma found in the soft tissues of children. (Refer to the PDQ summaries on Adult Soft Tissue Sarcoma Treatment; Childhood Soft Tissue Sarcoma Treatment; and Ewing Family of Tumors Treatment for more information.)

If your child has symptoms of a sarcoma, your child?s doctor may order x-rays and other tests. Your child?s doctor may also cut out a small piece of tissue and have it looked at under the microscope to see if there are any cancer cells. This is called a biopsy.

Your child?s chance of recovery (prognosis) and choice of treatment depend on where the cancer is located, how far it has spread, how the cells look under the microscope (histology), the type of therapy administered, how much of the cancer can be removed by surgery, and the child's age.

Stage Explanation

Stages of childhood rhabdomyosarcoma

Once childhood rhabdomyosarcoma is found, more tests will be done to find out if the cancercells have spread to other parts of the body. This is called staging. Your doctor needs to know how far the cancer has spread to plan treatment.

There are several staging systems for childhood rhabdomyosarcoma. The treatment options in this summary are based on size, location, and how far and where the cancer has spread.

Stage 1

Cancer is found in the bones around the eye, the head and neck, near the sex organs, or near the liver, gallbladder and bile ducts.

Stage 2

Cancer is located in 1 area (but in none of the areas in Stage 1), is 2 inches or smaller in size, and has not spread to the lymph nodes.

Stage 3

Cancer is located in 1 area (but in none of the areas in Stage 1), is greater than 2 inches (5 cm) in size, and may have spread to the lymph nodes.

Stage 4

Cancer has spread to other areas of the body at the time of diagnosis.

Recurrent

Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back in the area where it started or in another part of the body.

Treatment Option Overview

How childhood rhabdomyosarcoma is treated

There are treatments for all patients with childhood rhabdomyosarcoma. Three types of treatment are used, most often in combination with each other:

• Surgery

• Chemotherapy (using drugs to kill cancercells)

• Radiation therapy (using high-energy x-rays or other high-energy rays to kill cancer cells)

Surgery is a common treatment for rhabdomyosarcoma. Depending on where the cancer is, your child?s doctor will take out as much of the cancer as possible, along with some of the normal tissue around it. If the cancer is too large to remove or in a place where it cannot be removed, surgery may be limited to taking out only a small piece of the cancer (biopsy). Surgery is usually followed by chemotherapy and radiation therapy. Sometimes a second surgery is done to remove cancer that remains after these treatments.

Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by mouth in the form of a pill, or it may be put into the body by a needle in a vein or muscle. Chemotherapy is called a systemic treatment because the drugs enter the bloodstream, travel through the body, and can kill cancer cells throughout the body.

Radiation therapy uses high-energy x-rays to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external radiation therapy) or from putting materials that produce radiation (radioisotopes) through thin plastic tubes in the area where the cancer cells are found (internal radiation therapy). Clinical trials are testing radiation given in several small doses per day (hyperfractionated radiation therapy). Clinical trials for patients with head and neck rhabdomyosarcoma are also testing intensity-modulated radiation therapy, a type of 3-dimensional radiation that uses different strengths and angles to reduce damage to healthy tissue near the tumor.

High-dose chemotherapy with stem cell transplant is being studied for rhabdomyosarcoma. This is a method 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. When the patient's own stem cells are used, it is called an autologous stem cell transplant.

Treatment by stage

Treatment for childhood rhabdomyosarcoma depends on where the cancer is, how far it has spread, and what the cancer cells look like under a microscope.

Your child may receive treatment that is considered standard based on its effectiveness in a number of patients in past studies, or you may choose to have your child go into a clinical trial. Not all patients are cured with standard therapy and some standard treatments may have more side effects than are desired. For these reasons, clinical trials are designed to test new treatments and to find better ways to treat cancer patients. A large cooperative group clinical trial comparing new treatments with standard treatments is ongoing in most parts of the country for all stages of rhabdomyosarcoma. If you want more information, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.

Previously Untreated (Stages 1-4) Childhood Rhabdomyosarcoma

If the cancer can be removed, your child?s treatment will probably be surgery followed by chemotherapy. Radiation therapy may also be given if there is cancer left following surgery. If the cancer cannot be removed with surgery, your child?s treatment will probably be chemotherapy plus radiation therapy, followed, if needed, by surgery. Clinical trials are testing new chemotherapy drugs, new ways of giving radiation therapy, and autologous stem cell transplant following high-dose chemotherapy (for patients whose cancer has spread to other parts of the body).

Recurrent Childhood Rhabdomyosarcoma

Your child?s treatment depends on how much of the cancer can be removed by surgery, where the cancer came back, and the treatment your child received before. Your child?s treatment may be chemotherapy.Clinical trials are testing new chemotherapy drugs and autologous stem cell transplant.

Changes to This Summary (08/02/2007)

The PDQcancer 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.

Editorial changes were made to this summary.

To Learn More

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For more information, U.S. residents may call the National Cancer Institute's (NCI's) Cancer Information Service toll-free at 1-800-4-CANCER (1-800-422-6237) Monday through Friday from 9:00 a.m. to 4:30 p.m. Deaf and hard-of-hearing callers with TTY equipment may call 1-800-332-8615. The call is free and a trained Cancer Information Specialist is available to answer your questions.

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The NCI Web site provides online access to information on cancer, clinical trials, and other Web sites and organizations that offer support and resources for cancer patients and their families. There are also many other places where people can get materials and information about cancer treatment and services. Local hospitals may have information on local and regional agencies that offer information about finances, getting to and from treatment, receiving care at home, and dealing with problems associated with cancer treatment.

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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), TTY at 1-800-332-8615.

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), TTY at 1-800-332-8615.

The PDQ database contains listings of cancer health professionals and hospitals with cancer programs.

Because cancer in children and adolescents is rare, the majority of children with cancer are treated by health professionals specializing in childhood cancers, at hospitals or cancer centers with special facilities to treat them. The PDQ database contains listings of health professionals who specialize in childhood cancer and listings of hospitals with cancer programs. For help locating childhood cancer health professionals or a hospital with cancer programs, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.