Childhood Rhabdomyosarcoma Treatment (PDQ®)–Patient Version

SOFT TISSUE SARCOMA

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General Information About Childhood Rhabdomyosarcoma

KEY POINTS

Childhood rhabdomyosarcoma is a disease in which malignant (cancer) cells form in muscle tissue.
Certain genetic conditions increase the risk of childhood rhabdomyosarcoma.
A sign of childhood rhabdomyosarcoma is a lump or swelling that keeps getting bigger.
Diagnostic tests and a biopsy 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.

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, but can occur anywhere in the body. It is the most common type of rhabdomyosarcoma.
Alveolar: This type occurs most often in the arms or legs, chest, abdomen, genital organs, or anal area.
Anaplastic: This is the least common type of rhabdomyosarcoma in children.

See the following PDQ treatment summaries for information about other types of 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 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 diagnostic tests that are done depend in part on where the cancer forms. 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, pelvis, or lymph nodes, 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.
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Computed tomography (CT) scan of the abdomen. The child lies on a table that slides through the CT scanner, which takes x-ray pictures of the inside of the abdomen.
MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas of the body, such as the skull, brain, and lymph nodes. This procedure is also called nuclear magnetic resonance imaging (NMRI).
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Magnetic resonance imaging (MRI) of the abdomen. The child lies on a table that slides into the MRI scanner, which takes pictures of the inside of the body. The pad on the child’s abdomen helps make the pictures clearer.
PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
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Positron emission tomography (PET) scan. The child lies on a table that slides through the PET scanner. The head rest and white strap help the child lie still. A small amount of radioactive glucose (sugar) is injected into the child's vein, and a scanner makes a picture of where the glucose is being used in the body. Cancer cells show up brighter in the picture because they take up more glucose than normal cells do.
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 with cancer and is detected by a scanner.
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Bone scan. A small amount of radioactive material is injected into the child's vein and travels through the blood. The radioactive material collects in the bones. As the child lies on a table that slides under the scanner, the radioactive material is detected and images are made on a computer screen.
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.
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Bone marrow aspiration and biopsy. After a small area of skin is numbed, a bone marrow needle is inserted into the child’s hip bone. Samples of blood, bone, and bone marrow are removed for examination under a microscope.
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 diagnosingrhabdomyosarcoma.

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.
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.

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.
FISH (fluorescence in situ hybridization): A laboratory test used to look at genes or chromosomes in cells and tissues. Pieces of DNA that contain a fluorescent dye are made in the laboratory and added to cells or tissues on a glass slide. When these pieces of DNA attach to certain genes or areas of chromosomes on the slide, they light up when viewed under a microscope with a special light. This type of test is used to find certain gene changes.
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:

The patient's age.
Where in the body the tumor started.
The size of the tumor at the time of diagnosis.
Whether the tumor has been completely removed by surgery.
The type of rhabdomyosarcoma (embryonal, alveolar, or anaplastic).
Whether there are certain changes in the genes.
Whether the tumor had spread to other parts of the body at the time of diagnosis.
Whether the tumor was in the lymph nodes at the time of diagnosis.
Whether the tumor responds to chemotherapy and/or radiation therapy.

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

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

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Stages of Childhood Rhabdomyosarcoma