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Rhabdomyosarcoma

Rhabdomyosarcoma is a disease in which malignant cancer cells begin growing in muscle tissue anywhere in the body. 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 (skeletal) muscle. It can occur anywhere in the body.

It is a curable disease in the majority of children, with more than 60% of children still disease-free 5 years after diagnosis.

There are several types of sarcoma found in children and young adults. The cancer cells must be looked at under a microscope to tell the type of sarcoma. Rhabdomyosarcoma is the most common type of sarcoma found in the soft tissues of children. The most common sites are the head/neck, the genitourinary tract and the extremities.

If your child has symptoms of a sarcoma (pain or swelling), your child's doctor may order x-rays and other tests. Your child's doctor may then refer you to a pediatric oncologist. If the oncologist suspects rhabdomyosarcoma, they will refer you to an oncologic surgeon who will 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), and how much of the cancer can be removed during the initial surgery.

Stages of childhood rhabdomyosarcoma

Once childhood rhabdomyosarcoma is diagnosed, more tests will be done to find out if the cancer cells 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 appropriate treatment.

There are several staging systems for childhood rhabdomyosarcoma. The treatment options in this statement are based on size, location of primary tumor, how far and to where the cancer has spread (clinical staging).

Stage 1

Cancer is found localized (not spread) in areas such as around the eye, in the head and/or neck, genitourinary region (but not in the bladder or prostate), or biliary tract.

Stage 2

Cancer is located in only one area (but in none of the areas in Stage 1), is smaller in size than 2 inches across (5 cm), and has not spread to the lymph nodes.

Stage 3

Cancer is located in one area (but not one of the areas in Stage 1), is greater in size than 2 inches across (5 cm), and/or has spread to the lymph nodes found near the primary tumor.

Stage 4

Cancer has spread beyond the primary tumor site and is found in more than one place 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.

How childhood rhabdomyosarcoma is treated

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

  • surgery
  • radiation therapy (using high-dose x-rays or other high-energy rays to kill cancer cells)
  • chemotherapy (using drugs to kill cancer cells)

Usually treatment entails complete removal of the tumor with surgery (if possible), followed by chemotherapy. This is followed by "second-look surgery," which means that scans are done to see if chemotherapy has shrunk the tumor enough to have another attempt at removal. Then the child is given radiation therapy and additional chemotherapy.

Surgery is a common treatment for rhabdomyosarcoma. Depending on where the cancer is, the oncologic surgeon will take out as much of the cancer as possible, along with some of the normal tissue around it. If the cancer is in a place where it cannot be removed, surgery may be limited to taking out only a small piece of the cancer (biopsy).

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).

Bone marrow transplantation is a treatment being studied for recurrent rhabdomyosarcoma. Sometimes rhabdomyosarcoma becomes resistant to treatment with standard doses of radiation therapy or chemotherapy. Very high doses of chemotherapy may then be used to treat the cancer. Because the high doses of chemotherapy can destroy the bone marrow (where all the blood cells are produced) marrow is taken out before treatment. The marrow is then frozen and high-dose chemotherapy with or without radiation therapy is given. The marrow that was taken out is then thawed and given back through a needle in a vein to replace the marrow that was destroyed. This type of transplant is called an autologous transplant. Sometimes the source of cells is peripheral blood stem cells rather than bone marrow; your child's doctor will explain this process if it is needed.

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 the microscope (the histologic subtype).

Your child may receive treatment that is considered standard based on its effectiveness in patients in past clinical trials, or you may choose to have your child go into a clinical trial. Clinical trials are designed to compare potentially better treatments with treatment that is currently accepted as standard of care in hopes of finding better ways to treat cancer patients. A large cooperative group clinical trial is going on in most parts of the country for all stages of rhabdomyosarcoma. The good news is that Children's Oncology Group clinical trials are closely overseen by a data monitoring committee, so that in real time, if one treatment is found to be better than another, the treating physicians are notified immediately, and your child's treatment will be changed right away if necessary.

Previously untreated (stages 1-4) childhood rhabdomyosarcoma

If the cancer can be completely removed, your child's treatment will probably be surgery followed by chemotherapy. Radiation therapy may be given if there is cancer left following surgery or if the cancer is of a specific histologic subtype. If the cancer cannot be removed with surgery or it is near an important organ (e.g., the eye or bladder), 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 bone marrow transplantation 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. However, the long-term prognosis in these patients is guarded. Your child's treatment will most likely be chemotherapy. Clinical trials are testing new chemotherapy agents and autologous bone marrow transplantation.

Cleveland Clinic Children's

Here at Cleveland Clinic Children's, we specialize in the care of children and young adults with cancer. We use a team approach that cares for all members of the family, recognizing that this is a very difficult time for all of them. Our goal is to have our patients get through therapy with as normal a life as possible, and to emerge from therapy knowledgeable about how to maintain their health.

Should you have more questions about the child or young adult in your life with cancer, please contact Cleveland Clinic Cancer Answer line at 216.444.3024 or 800.223.2273, extension 4-3024. Your question will be forwarded to the Chair of the Pediatric Oncology Department.

We wish you and your child well.

For other websites regarding childhood cancer:

Source: National Institutes of Health, National Cancer Institute, Children's Oncology Group

© 1995-2009 The Cleveland Clinic Foundation. All rights reserved.

This information is provided by the Cleveland Clinic Health System and is not intended to replace the medical advice of your doctor or health care provider. Please consult your health care provider for advice about a specific medical condition. Rev. 1/03


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