Soft Tissue Sarcoma

General Information | Treatment Options | Resources

 

Treatment
  • Overview
  • Standard Treatment
  • Clinical Trials
  • Treatment By Stage

There are different types of treatment for patients with childhood soft tissue sarcoma.

Different types of treatments are available for patients with childhood soft tissue sarcoma. 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 childhood soft tissue sarcoma should have their treatment planned by a team of doctors with expertise in treating cancer in children.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric doctors who are experts in treating children with cancer and who specialize in certain areas of medicine. These may include a pediatric surgeon with special training in the removal of soft tissue sarcomas. The following specialists may also be included:

  • Radiation oncologist.
  • Pediatric hematologist.
  • Pediatric nurse specialist.
  • Rehabilitation specialist.
  • Psychologist.
  • Social worker.

Some cancer treatments cause side effects months or years after treatment has ended.

Some cancer treatments cause side effects that continue or appear months or years after cancer treatment has ended. These are called late effects. Late effects of cancer treatment 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 possible late effects caused by some treatments. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer for more information.)

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

Seven types of standard treatment are used:

Surgery

Surgery to completely remove the soft tissue sarcoma is done whenever possible. If the tumor is very large, radiation therapy or chemotherapy may be given first, to make the tumor smaller and decrease the amount of tissue that needs to be removed during surgery. The following types of surgery may be used:

  • Wide local excision: Removal of the tumor along with some normal tissue around it.
  • Amputation: Surgery to remove part or all of a limb or appendage, such as the arm or hand.
  • Limb-sparing surgery: Removal of the tumor in an arm or leg without amputation, so the use and appearance of the limb is saved. Radiation therapy or chemotherapy may be given first to shrink the tumor. The tumor is then removed in a wide local excision. Tissue and bone that are removed may be replaced with a graft using tissue and bone taken from another part of the patient's body, or with an implant such as artificial bone.
  • Lymphadenectomy: Removal of the lymph nodes that contain cancer.
  • Thoracotomy: An operation to open the chest. This may be done to remove cancer that has spread to the lungs.

A second surgery may be needed to:

  • remove any remaining cancer cells.
  • check the area around where the tumor was removed for cancer cells and then remove them.

Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given radiation therapy or chemotherapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to increase the chances of a cure, 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 keep 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. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

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 spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Hormone therapy

Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances produced by glands in the body and circulated in the bloodstream. Some hormones can cause certain cancers to grow. If tests show that the cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy are used to reduce the production of hormones or block them from working. Antiestrogens (drugs that block estrogen) may be used to treat childhood soft tissue sarcoma.

Watchful waiting

Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change. Watchful waiting may be done when complete removal of the tumor is not possible, no other treatments are available, and the tumor does not place any vital organs in danger.

Nonsteroidal anti-inflammatory drugs

Nonsteroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin, ibuprofen, and naproxen) that are commonly used to decrease fever, swelling, pain, and redness. In the treatment of soft tissue sarcomas, an NSAID called sulindac may be used to help block the growth of cancer cells.

Liver transplant

The liver is removed and replaced with a healthy one from a donor.

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.

Targeted drug therapy

Targeted therapy is type of treatment that uses drugs or other substances to find and attack specific cancer cells without harming normal cells. Imatinib (Gleevec) is a new type of targeted therapy called a tyrosine kinase inhibitor. It finds and blocks an abnormal protein on cancer cells that causes them to divide and 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 clinical trials database.

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Nonmetastatic Childhood Soft Tissue Sarcoma

For treatment of fibrosarcoma or hemangiopericytoma

If an infant or young child has fibrosarcoma or hemangiopericytoma, treatment will be surgery whenever possible. Other treatments may include chemotherapy to reduce the size of the tumor, followed by surgery.

If an older child or adolescent has fibrosarcoma or hemangiopericytoma, treatment will be surgery whenever possible. Other treatments may include the following:

  • Chemotherapy followed by radiation therapy, surgery, or both.
  • A clinical trial of internal radiation therapy.
  • A clinical trial of radiation therapy given during surgery.

For treatment of desmoid tumor

Treatment of desmoid tumor will be surgery whenever possible.

  • If surgery to completely remove the tumor is not possible, treatment may include the following:
    • External radiation therapy to shrink the tumor before surgery.
    • Internal radiation therapy to kill any remaining tumor cells after surgery.
    • A nonsteroidal anti-inflammatory drug (NSAID).
    • Antiestrogen therapy.
    • Chemotherapy.
  • Watchful waiting.
  • A clinical trial of targeted drug therapy with imatinib (Gleevec).

For treatment of malignant peripheral nerve sheath tumor, liposarcoma, synovial sarcoma, extraosseous osteosarcoma, extraosseous chondrosarcoma, malignant fibrous histiocytoma, leiomyosarcoma, or epithelioid sarcoma

Treatment of these tumor types will be surgery whenever possible. Other treatments may include the following:

  • Chemotherapy followed by radiation therapy, surgery, or both.
  • A clinical trial of internal radiation therapy.
  • A clinical trial of radiation therapy given during surgery.

Synovial sarcoma may also be treated with surgery followed by chemotherapy. Malignant peripheral nerve sheath tumor may be treated with radiation therapy after surgery.

For treatment of alveolar soft part sarcoma

Treatment of alveolar soft part sarcoma will be surgery whenever possible. Other treatments may include the following:

  • Radiation therapy after surgery.
  • A clinical trial of chemotherapy after surgery.

For treatment of desmoplastic small round cell tumor

Treatment of desmoplastic small round cell tumor will be surgery whenever possible. Other treatments may include chemotherapy and radiation therapy after surgery.

For treatment of clear cell sarcoma of soft parts

Treatment of clear cell sarcoma of soft parts will be surgery whenever possible. Other treatments may include radiation therapy after surgery.

For treatment of hemangioendothelioma

Treatment of hemangioendothelioma in children aged one year and older may include the following:

  • Surgery to remove the tumor.
  • Treatment for blood clotting disorders.
  • Liver transplant (if the tumor is in the liver).

Treatment of hemangioendothelioma in children younger than one year may include the following:

  • Watchful waiting as some tumors will disappear without treatment.
  • Treatment for blood clotting disorders.
  • Surgery to remove the tumor if there are symptoms.

For treatment of angiosarcoma and lymphangiosarcoma

Treatment of angiosarcoma and lymphangiosarcoma may include the following:

  • Surgery to completely remove the tumor.
  • Radiation therapy after surgery.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with nonmetastatic childhood soft tissue sarcoma.

Metastatic Childhood Soft Tissue Sarcoma

Treatment of metastatic childhood soft tissue sarcoma may include the following:

  • Combination chemotherapy, radiation therapy, and surgery (thoracotomy) to remove cancer that has spread to the lungs.
  • A clinical trial of combination chemotherapy and granulocyte colony-stimulating factor (substance that helps the body increase the number of white blood cells).

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with metastatic childhood soft tissue sarcoma.

Recurrent and Progressive Childhood Soft Tissue Sarcoma

Treatment of recurrent or progressive childhood soft tissue sarcoma may include the following:

  • Surgery followed by radiation therapy, if not already given.
  • Surgery to remove the arm or leg with cancer, if radiation therapy was already given.
  • Surgery to remove cancer that has spread to the lungs.
  • A clinical trial of chemotherapy or targeted drug therapy with imatinib (Gleevec).
  • A clinical trial of a new treatment.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood soft tissue sarcoma.

 

 

Cancer information from the NCI PDQ service