National Cancer Institute


Posted Date: Apr 27, 2014

Expert-reviewed information summary about the treatment of childhood rhabdomyosarcoma.

Childhood Rhabdomyosarcoma Treatment

General Information About Childhood Rhabdomyosarcoma

Key Points for this Section

  • Childhood rhabdomyosarcoma is a disease in which malignant (cancer) cells form in muscle tissue.
  • Certain genetic conditions increase the risk of childhood rhabdomyosarcoma.
  • A possible sign of childhood rhabdomyosarcoma is a lump or swelling that keeps getting bigger.
  • Tests that examine the area where symptoms have occurred are used to detect (find) and diagnose childhood rhabdomyosarcoma.
  • Certain factors affect prognosis (chance of recovery) and treatment options.

Childhood rhabdomyosarcoma is a disease in which malignant (cancer) cells form in muscle tissue.

Rhabdomyo sarcoma 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:

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 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. Check with a doctor if your child has any of the following problems:

  • 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 where symptoms have occurred 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.
  • 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.
  • 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 to check for cancer cells. This is done by placing a needle between two bones in the spine and into the spinal column to remove a sample of CSF. This procedure is also called an LP or spinal tap.
  • Sentinel lymph node biopsy: The removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes. A sentinel lymph node biopsy is done to check whether rhabdomyosarcoma of the arms or legs has spread to the lymph nodes.
  • 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 find out what type it is. Because treatment depends on the type of rhabdomyosarcoma, biopsy samples should be checked by a pathologist who has experience in diagnosing rhabdomyosarcoma. The following tests may be done on the sample of tissue that is removed: 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.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 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.

Stages of Childhood Rhabdomyosarcoma

Key Points for this Section

  • After childhood rhabdomyosarcoma has been diagnosed, treatment is based on the stage of the cancer and whether all the cancer was removed by surgery.
  • There are three ways that cancer spreads in the body.
  • Cancer may spread from where it began to other parts of the body.
  • Staging of childhood rhabdomyosarcoma is done in three parts.
  • 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
    • Stage 2
    • Stage 3
    • Stage 4
  • The grouping system is based on whether the cancer has spread and whether all the cancer was removed by surgery:
    • Group I
    • Group II
    • Group III
    • Group IV
  • The risk group is based on the staging system and the grouping system.
    • Low-risk childhood rhabdomyosarcoma
    • Intermediate-risk childhood rhabdomyosarcoma
    • High-risk childhood rhabdomyosarcoma

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:

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:

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.

Tumor size compared to everyday objects; shows various measurements of a tumor compared to a pea, peanut, walnut, and lime
Pea, peanut, walnut, and lime show tumor sizes.

Stage 2

In stage 2, cancer is found in an "unfavorable" site (any one area not included in stage 1). The tumor is no larger than 5 centimeters and has not spread to lymph nodes.

Stage 3

In stage 3, cancer is found in an "unfavorable" site (any one area not included in stage 1) and one of the following is true:

  • The tumor is no larger than 5 centimeters 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 grouping system is based on whether the cancer has spread and whether all the cancer was removed by surgery:

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 found.

Group II

Group II is divided into groups IIA, IIB, and IIC.

  • IIA: Cancer was removed by surgery but cancer cells were seen when the tissue, taken from the edges of where the tumor was removed, was viewed under a microscope by a pathologist.
  • 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. Tissue was taken from the edges of where the tumor was removed. The tissue was checked under a microscope by a pathologist and cancer cells were seen.

Group III

Cancer was partly removed by biopsy or surgery but there is tumor remaining that can be seen with the eye. Cancer has not spread to distant parts of the body.

Group IV

Cancer had spread to distant parts of the body when the cancer was diagnosed.

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:

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 nearby lymph nodes. The following areas are "favorable" sites: Eye or area around the eye.Head or neck (but not in the tissue next to the brain and spinal cord).Gallbladder and bile ducts.In the testes, vagina, or uterus.
    • Eye or area around the eye.
    • Head or neck (but not in the tissue next to the brain and spinal cord).
    • Gallbladder and bile ducts.
    • In the testes, vagina, or uterus.
  • 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 nearby 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 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

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

Key Points for this Section

  • There are different types of treatment for patients with childhood rhabdomyosarcoma.
  • Children with rhabdomyosarcoma should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
  • Some cancer treatments cause side effects months or years after treatment has ended.
  • Three types of standard treatment are used:
    • Surgery
    • Radiation therapy
    • Chemotherapy
  • New types of treatment are being tested in clinical trials.
    • High-dose chemotherapy with stem cell transplant
    • Immunotherapy
    • Targeted therapy
  • Patients may want to think about taking part in a clinical trial.
  • Patients can enter clinical trials before, during, or after starting their cancer treatment.
  • Follow-up tests may be needed.

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:

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:

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 tissue around it, including the lymph nodes. 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 way the child will look.
  • 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.

In most children with rhabdomyosarcoma, it is not possible to remove all of the tumor by surgery.

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 after surgery to kill any cancer cells that are left. Radiation therapy may also be given. 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. Certain ways of giving radiation therapy can help keep radiation from damaging healthy tissue. These types of external radiation therapy include the following:

  • Conformal radiation uses a computer to create a 3-dimensional (3-D) picture of the tumor. The radiation beams are shaped to fit the tumor.
  • Intensity-modulated radiation therapy (IMRT) uses images created by a computer that show the size and shape of the tumor. Thin beams of radiation of different strengths are aimed at the tumor from many angles.
  • Fractionated stereotactic radiation therapy uses a rigid head frame attached to the skull to aim radiation directly to a tumor. This causes less damage to nearby healthy tissue. The total dose of radiation is divided into several small doses given over several days. This type of radiation therapy may be used for rhabdomyosarcoma of the head and neck. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy.
  • Proton-beam therapy is a type of high-energy, external radiation therapy that uses streams of protons (small, positively-charged particles of matter) to kill tumor cells.

Internal radiation therapy (brachytherapy) uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. Internal radiation therapy is used to treat cancer in areas such as the vagina, vulva, bladder, prostate, head, or neck.

The type and amount of radiation therapy and when it is given depends on the age of the child, the type of rhabdomyosarcoma, where in the body the tumor started, how much tumor remained after surgery, and whether there is tumor in the nearby lymph nodes.

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.

Every child treated for rhabdomyosarcoma should receive chemotherapy to decrease the chance the 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.

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. Targeted therapy with angiogenesis inhibitors is being studied to treat childhood rhabdomyosarcoma. Angiogenesis inhibitors keep blood vessels from forming in a tumor. This causes the tumor to starve and stop growing or to shrink. Monoclonal antibodies and kinase inhibitors are two types of antiangiogenic agents used in clinical trials for 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 used in clinical trials for childhood rhabdomyosarcoma attach to and block substances that cause new blood vessels to form in tumors. Monoclonal antibodies may be used with chemotherapy as adjuvant therapy.

Kinase inhibitors stop cells from dividing and may prevent the growth of new blood vessels that tumors need to grow.

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 child's 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

Previously Untreated Childhood Rhabdomyosarcoma

The treatment of childhood rhabdomyosarcoma often includ

English
News
Appears to increase risk after liver transplant in younger patients, those with C2 monitoring

Jul 1, 2010 - Immunosuppressive treatment with cyclosporine A, rather than tacrolimus, with dose level monitoring two hours post-dosing or in patients age 50 or younger appears to have a significant association with the development of de novo cancer after liver transplantation, according to research published in the July issue of Liver Transplantation.



OncoLink Treatment Binder

Seleccione los efectos secundarios y formas de ligante de tratamiento de su paciente

Más información



Blogs and Web Chats

OncoLink Blogs give our readers a chance to react to and comment on key cancer news topics and provides a forum for OncoLink Experts and readers to share opinions and learn from each other.




OncoLink OncoPilot

Frente a un nuevo diagnóstico de cáncer o de cambiar el curso de su tratamiento actual? Deje que nuestro personal de enfermería cáncer que ayudan a pasar!

Más información