|NCI/PDQ® Patients: Childhood Soft Tissue Sarcoma|
|National Cancer Institute|
| Last Modified: October 18, 2006
Childhood soft tissue sarcoma is a disease in which cancer (malignant) cells begin growing in soft tissue in the body. The soft tissues include muscles, tendons (bands of fiber that connect muscles to bones), fibrous (connective) tissues, fat, blood vessels, nerves, and synovial tissues (tissues around joints). Soft tissues connect, support, and surround other body parts and organs.
Soft tissue sarcomas are rare in children and adolescents. If a patient has symptoms of a soft tissue sarcoma, the doctor may order x-rays and other tests. The 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. If cancer cells are found, the doctor may remove as much tumor as safely possible, along with some healthy tissue around it, during the same surgery. Some soft tissue sarcomas may be completely removed by surgery.
There are many different kinds of soft tissue sarcoma, depending on the soft tissue where the cancer begins. Rhabdomyosarcoma is the most common type of childhood soft tissue sarcoma. It begins in muscles around the bone and can be found anywhere in the body. (Refer to the PDQ® summaries on Childhood Rhabdomyosarcoma Treatment and Adult Soft Tissue Sarcoma Treatment for more information.) The soft tissue sarcomas that affect young patients include tumors of the smooth muscle, connective tissue, blood and lymphatic vessels, and the peripheral nervous system.
Soft tissue sarcomas may develop in any part of the body, but in young patients, they are most commonly found in the trunk, arms, and legs. The first symptom may be a solid mass or lump. If the mass interferes with a function of the body, it may cause other symptoms. Soft tissue sarcoma rarely causes fever, weight loss, or night sweats.
Soft tissue sarcoma is more likely to develop in people who have specific genetic conditions, such as Li-Fraumeni syndrome, who have previously received radiation therapy, or who have the Epstein-Barr virus with acquired immune deficiency syndrome (AIDS).
Once childhood soft tissue sarcoma is found, more tests will be done to find out if the cancer cells have spread to other parts of the body. This is called staging. The doctor needs to know the stage of the cancer to plan treatment.
There are several staging systems for childhood soft tissue sarcoma, but no single staging system applies to all types of this cancer. The treatment options in this summary are based on whether the cancer has spread or the amount of tumor left after surgery. The 3 general stages of soft tissue sarcoma are nonmetastatic, metastatic, and recurrent.
The cancer has been partly or completely removed in surgery and has not spread to other parts of the body.
The cancer has spread from where it started to other parts of the body.
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.
There are treatments for all patients with childhood soft tissue sarcoma. Three types of treatment are used:
Radiation therapy uses high-energy rays to kill cancer cells and shrink tumors. Radiation may be given before surgery or following surgery (if the surgeon is unable to remove adequate tissue surrounding the tumor). Radiation may come from a machine outside the body (external radiation therapy) or from putting materials that produce radiation (radioisotopes) through thin plastic tubes into the area where the cancer cells are found (internal radiation therapy).
Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be given before or after surgery. 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.
In addition, biologic therapy is being tested in clinical trials for soft tissue sarcoma. Biologic therapy is used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.
Some cancer treatments cause side effects that continue or appear years after cancer treatment has ended. These are called late effects. It is important that parents of children who are treated for cancer know about the possible late effects caused by certain treatments. After several years, some patients develop another form of cancer as a result of their treatment with chemotherapy and radiation. Refer to the PDQ® summary on Late Effects of Treatment for Childhood Cancer for more information. Clinical trials are ongoing to determine if lower doses of chemotherapy and radiation can be used.
Treatment for soft tissue sarcoma depends on where the cancer is, how far it has spread, and what the cancer cells look like under a microscope.
The patient may receive treatment that is considered standard based on its effectiveness in a number of patients in past studies, or the doctor may recommend that the patient enter a clinical trial. Not all patients are cured with standard therapy and some standard treatments may have unwanted side effects. For these reasons, clinical trials are designed to test new treatments and to find better ways to treat cancer patients. If you want more information about clinical trials for soft tissue sarcoma, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237); TTY at 1-800-332-8615.
Treatment depends on the type of soft tissue sarcoma.
Clinical trials are evaluating the effectiveness of biologic therapy in desmoid tumors.
If your older child or adolescent has fibrosarcoma or has hemangiopericytoma, or if your child has malignant peripheral nerve sheath tumor, liposarcoma, synovial sarcoma, malignant fibrous histiocytoma, leiomyosarcoma, or epithelioid sarcoma, treatment may be one of the following:
If your child has desmoplastic small round cell tumor, treatment will probably be surgery to remove as much of the tumor as possible, followed by chemotherapy and radiation therapy.
If your child has a vascular tumor, treatment will probably be surgery.
If your child has undifferentiated sarcoma, which is similar in some ways to rhabdomyosarcoma, treatment may include a clinical trial for rhabdomyosarcoma.
Treatment for recurrent childhood soft tissue sarcoma depends on the treatment your child received before, the part of the body where the cancer has come back, and your child's general condition. You may wish to have your child take part in a clinical trial. Treatment may be one of the following:
Editorial changes were made to this summary.
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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 new treatments, the risks involved, and how well they do or do not work. 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.
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