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), or bone. Rhabdomyosarcoma usually begins in muscles that are attached to bones and that help the body move. Rhabdomyosarcoma is the most common type of soft tissue sarcoma in children. It can begin 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, genital organs, or anal area. It usually occurs during the teen years.
• Anaplastic: This type rarely occurs in children.

See the following PDQ treatment summaries for more information about soft tissue sarcomas:

• Childhood Soft Tissue Sarcoma
• Adult Soft Tissue Sarcoma

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. Talk with your child’s doctor if you think your child may be at risk. Risk factors for rhabdomyosarcoma include having the following inherited diseases:

• Li-Fraumeni syndrome.
• Pleuropulmonary blastoma.
• Neurofibromatosis type 1 (NF1).
• Beckwith-Wiedemann syndrome.
• Costello syndrome.
• Noonan syndrome.

Children who had a high birth weight or were larger than expected at birth may have an increased risk of embryonal rhabdomyosarcoma.

In most cases, the cause of rhabdomyosarcoma is not known.

A sign of childhood rhabdomyosarcoma is a lump or swelling that keeps getting bigger.

Signs and symptoms may be caused by childhood rhabdomyosarcoma or by other conditions. The signs and symptoms that occur depend on where the cancer forms. Check with your child's doctor if your child has any of the following:

• 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.

Diagnostic tests and a biopsy 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, such as the chest. 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.
• 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.
• Lumbar puncture: A procedure used to collect cerebrospinal fluid (CSF) from the spinal column. This is done by placing a needle between two bones in the spine and into the CSF around the spinal cord and removing a sample of the fluid. The sample of CSF is checked under a microscope for signs of cancer cells. This procedure is also called an LP or spinal tap.

If these tests show there may be a rhabdomyosarcoma, a biopsy is done. A biopsy is the removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. Because treatment depends on the type of rhabdomyosarcoma, biopsy samples should be checked by a pathologist who has experience in diagnosing rhabdomyosarcoma.

One of the following types of biopsies may be used:

• Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluid using a thin needle.
• Core needle biopsy: The removal of tissue using a wide needle. This procedure may be guided using ultrasound, CT scan, or MRI.
• Open biopsy: The removal of tissue through an incision (cut) made in the skin.

The following tests may be done on the sample of tissue that is removed:

• Light 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: A test that uses antibodies to check for certain antigens in a sample of tissue. The antibody is usually linked to a radioactive substance or a dye that causes the tissue to light up under a microscope. This type of test may be used to tell the difference between different types of cancer.
• Immunocytochemistry: A test that uses antibodies to check for certain antigens in a sample of cells. The antibody is usually linked to a radioactive substance or a dye that causes the cells to light up under a microscope. This type of test may be used to tell the difference between different types of soft tissue sarcoma.
• Reverse transcription-polymerase chain reaction (RT-PCR) test: A laboratory test in which cells in a sample of tissue are studied using chemicals to look for certain changes in the structure or function of genes.
• Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.

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 width of the tumor when the cancer was diagnosed.
• Whether the tumor has spread to nearby lymph nodes or distant parts of the body.
• Whether there are certain changes in the genes.
• The type of rhabdomyosarcoma.
• Whether the tumor has been completely removed by surgery.
• Whether the tumor responds to chemotherapy and/or radiation therapy.
• The patient's age and general health.
• Whether the tumor has just been diagnosed or has recurred (come back).

For patients with recurrent cancer, prognosis and treatment depend on the following:

• Where in the body the tumor recurred (came back).
• Whether the tumor was treated with radiation therapy.
• The size of the tumor when the cancer was diagnosed.
• How much time passed between the end of cancer treatment and when the cancer recurred.

After childhood rhabdomyosarcoma has been diagnosed, treatment is based on the stage of the cancer and whether all the cancer was removed by surgery.

The process used to find out if cancer has spread within the tissue 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 find out 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.

Cancer can spread through tissue, the lymph system, and the blood:

• Tissue. The cancer spreads from where it began by growing into nearby areas.
• Lymph system. The cancer spreads from where it began by getting into the lymph system. The cancer travels through the lymph vessels to other parts of the body.
• Blood. The cancer spreads from where it began by getting into the blood. The cancer travels through the blood vessels to other parts of the body.

Cancer may spread from where it began to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they began (the primary tumor) and travel through the lymph system or blood.

• Lymph system. The cancer gets into the lymph system, travels through the lymph vessels, and forms a tumor (metastatic tumor) in another part of the body.
• Blood. The cancer gets into the blood, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

The metastatic tumor is the same type of cancer as the primary tumor. For example, if rhabdomyosarcoma spreads to the lung, the cancer cells in the lung are actually rhabdomyosarcoma cells. The disease is metastatic rhabdomyosarcoma, not lung cancer.

Staging of childhood rhabdomyosarcoma is done in three parts.

Childhood rhabdomyosarcoma is staged by using three different ways to describe the cancer:

• A staging system.
• A grouping system.
• A risk group.

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, the tumor is any size, has not spread to lymph nodes, and is found in only one of the following "favorable" sites:

• Eye or area around the eye.
• Head and neck (but not in the tissue next to the brain and spinal cord).
• Gallbladder and bile ducts.
• In the testes, vagina, or uterus.

Rhabdomyosarcoma that forms 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.

Figure

Pea, peanut, walnut, and lime show tumor sizes.

The grouping system is based on whether the cancer has spread and whether all the cancer was removed by surgery:

The risk group is based on the staging system and the grouping system.

The risk group describes the chance that rhabdomyosarcoma will recur (come back). Every child treated for rhabdomyosarcoma should receive chemotherapy to decrease the chance cancer will recur. The type of anticancer drug, dose, and the number of treatments given depends on whether the child has low-risk, intermediate-risk, or high-risk rhabdomyosarcoma. The following risk groups are used:

There are different types of treatment for patients with childhood 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:

• Pediatrician.
• Pediatric surgeon.
• Radiation oncologist.
• Pediatric hematologist.
• Pediatric nurse specialist.
• Geneticist or cancer genetics risk counselor.
• Social worker.
• Rehabilitation specialist.
• Psychologist.

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:

• 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 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:

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 website.

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.

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 child's condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Previously Untreated Childhood Rhabdomyosarcoma

The treatment of childhood rhabdomyosarcoma often includes surgery, radiation therapy, and chemotherapy. The order that these treatments are given depends on where in the body the tumor started, the size of the tumor, the type of tumor, and whether the tumor has spread to lymph nodes or other parts of the body. See the Treatment Option Overview section of this summary for more information about surgery, radiation therapy, and chemotherapy used to treat children with rhabdomyosarcoma.

Rhabdomyosarcoma of the brain and head and neck

• For tumors of the brain: Treatment may include surgery to remove the tumor, radiation therapy, and chemotherapy.
• For tumors of the head and neck that are in or near the eye: Treatment may include 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 are near the brain and spinal cord but not in or near the eye: Treatment may include radiation therapy and chemotherapy.
• For tumors of the head and neck that cannot be removed by surgery: Treatment may include chemotherapy and radiation therapy.
• For tumors of the larynx (voice box): Treatment may include chemotherapy and radiation therapy. Surgery to remove the larynx is usually not done, so that the voice is not harmed.

Rhabdomyosarcoma of the arms or legs

• Surgery to remove the tumor. If the tumor was not completely removed, a second surgery to remove the tumor may be done.
• For tumors of the hand or foot, radiation therapy and chemotherapy may be given. The tumor may not be removed because the function of the hand or foot would be lessened.
• Lymph node dissection (one or more lymph nodes are removed and a sample of tissue is checked under a microscope for signs of cancer).

  -

  For tumors in the arms, lymph nodes near the tumor and in the armpit area are removed.

  -

  For tumors in the legs, lymph nodes near the tumor and in the groin area are removed.

• Chemotherapy.
• Radiation therapy.

Rhabdomyosarcoma of the chest, abdomen, or pelvis

• For tumors in the chest or abdomen (including the chest wall or abdominal wall): Surgery (wide local excision) may be done. If the tumor is large, chemotherapy, and sometimes radiation therapy, is given to shrink the tumor before surgery.
• For tumors of the pelvis: Surgery (wide local excision) may be done. If the tumor is large, chemotherapy, and sometimes radiation therapy, is given to shrink the tumor before surgery. Some pelvic tumors may be treated with biopsy, rather than wide local excision, followed by radiation therapy.
• For tumors of the diaphragm: A biopsy of the tumor is followed by chemotherapy and radiation therapy to shrink the tumor. Surgery may be done later to remove any remaining cancer cells.
• For tumors of the gallbladder or bile ducts: Surgery is done to remove as much of the tumor as possible, followed by chemotherapy and radiation therapy.
• 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 and radiation therapy.

Rhabdomyosarcoma of the kidney

• Surgery to remove as much of the tumor as possible.

Rhabdomyosarcoma of the bladder and prostate

• For tumors that are only at the top of the bladder: Surgery (wide local excision) is done.
• For tumors of the prostate or bladder (other than the top of the bladder):

  -

  Chemotherapy and radiation therapy are given first to shrink the tumor. If cancer cells remain after chemotherapy and radiation therapy, the tumor is removed by surgery. Surgery may include removal of the prostate, part of the bladder, or pelvic exenteration without removal of the rectum. (This may include removal of the lower colon and bladder. In girls, the cervix, vagina, ovaries, and nearby lymph nodes may be removed).

  -

  Chemotherapy is given first to shrink the tumor. Surgery to remove the tumor, but not the bladder or prostate, is done. Internal radiation therapy may be given after surgery.

Rhabdomyosarcoma of the area near the testicles

• Rhabdomyosarcoma of the testicular area is usually treated with surgery to remove the testicle and spermatic cord.
• The lymph nodes in the back of the abdomen may be checked for cancer, especially if the lymph nodes are enlarged or the child is older than 9 years. Radiation therapy may be given if the tumor cannot be completely removed by surgery. CT scans may be done every 3 months after surgery to see if the cancer is growing in nearby lymph nodes.

Rhabdomyosarcoma of the vulva, vagina, uterus or ovary

• For tumors of the vulva and vagina: Treatment may include chemotherapy followed by surgery to remove the tumor. Internal or external radiation therapy may be given after surgery.
• For tumors of the uterus: Treatment may include chemotherapy with or without radiation therapy. Sometimes surgery may be needed to remove any remaining cancer cells.
• For tumors of the cervix: Treatment may include chemotherapy followed by surgery to remove any remaining tumor.
• For tumors of the ovary: Treatment may include combination chemotherapy followed by surgery to remove any remaining tumor.

Metastatic rhabdomyosarcoma

• Radiation therapy may be given for tumors that have spread to the brain, spinal cord, or lungs.
  Treatment is also given to the site where the tumor first formed.
  The following treatment is being studied for rhabdomyosarcoma:

  • A clinical trial of immunotherapy.

Check the list of NCI-supported 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. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.

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:

• Chemotherapy with one or more anticancer drugs.
• Surgery.
• Radiation therapy.
• A clinical trial of new anticancer drugs .
• A clinical trial of high-dose chemotherapy followed by stem cell transplant using the patient's own stem cells.

Check the list of NCI-supported 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. Talk with your child's doctor about clinical trials that may be right for your child. General information about clinical trials is available from the NCI website.

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This PDQ cancer information summary has current information about the treatment of childhood rhabdomyosarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.

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