Childhood Soft Tissue Sarcoma

What is childhood soft tissue sarcoma?

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 tumour 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 summaries on Childhood Rhabdomyosarcoma Treatment and Adult Soft Tissue Sarcoma Treatment for more information.) The soft tissue sarcomas that affect young patients include tumours 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).

Soft tissue sarcomas are classified according to the type of soft tissue they resemble. The types of soft tissue sarcoma include:

Tumours of fibrous (connective) tissue:

Fibrohistiocytic tumours:

Fat tissue tumours:

Smooth muscle tumours:

Blood and lymph vessel tumours:

Peripheral nervous system tumours:

Bone and cartilage tumours:

Tumours with more than one type of tissue:

Tumours of unknown origin:

The chance of recovery (prognosis) and choice of treatment depend on the type, location, and stage of the tumour and the age, size, stage of development, and general health of the patient.

Stage Explanation

Stages of childhood soft tissue sarcoma

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 tumour left after surgery. The 3 general stages of soft tissue sarcoma are nonmetastatic, metastatic, and recurrent.
Nonmetastatic childhood soft tissue sarcoma

The cancer has been partly or completely removed in surgery and has not spread to other parts of the body.
Metastatic childhood soft tissue sarcoma

The cancer has spread from where it started to other parts of the body.
Recurrent soft tissue sarcoma

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.

Treatment Option Overview

How childhood soft tissue sarcoma is treated

There are treatments for all patients with childhood soft tissue sarcoma. Three types of treatment are used:

Surgery (taking out the cancer in an operation).

Radiation therapy (using high-dose x-rays or other high-energy rays to kill cancer cells).

Chemotherapy (using drugs to kill cancer cells).

Surgery is the standard treatment for soft tissue sarcoma. The surgeon will remove as much of the cancer as possible, along with some of the normal tissue around it.

Radiation therapy uses high-energy rays to kill cancer cells and shrink tumours. Radiation may be given before surgery or following surgery (if the surgeon is unable to remove adequate tissue surrounding the tumour). 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 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 by stage

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.

Nonmetastatic Childhood Soft Tissue Sarcoma

Treatment depends on the type of soft tissue sarcoma.

If your infant or young child has fibrosarcoma or hemangiopericytoma or if your child has desmoid tumour or angiomatoid malignant fibrous histiocytoma, treatment may be one of the following:

1. Surgery to remove all of the cancer. Sometimes a second operation must be done to be sure that all the tumour has been removed, or if the tumour comes back following treatment.
2. Surgery to remove the cancer followed by radiation therapy (if a second surgery is not possible).
3. Chemotherapy to reduce the tumour size, followed by surgery.

Clinical trials are evaluating the effectiveness of new combinations of chemotherapy drugs.

If your child has desmoid tumour, treatment may be one of the following:

1. Surgery to remove all of the cancer.
2. Radiation therapy given before or after surgery, nonsteroidal antiinflammatory agents, antioestrogens, and chemotherapy (if complete removal of the tumour is not possible).
3. Patients may undergo close monitoring when no other treatment alternatives are available and the tumour does not place any vital organs in danger (if complete removal of the tumour is not possible or if the tumour comes back following treatment).

Clinical trials are evaluating the effectiveness of biologic therapy in desmoid tumours.

If your older child or adolescent has fibrosarcoma or has hemangiopericytoma, or if your child has malignant peripheral nerve sheath tumour, liposarcoma, synovial sarcoma, malignant fibrous histiocytoma, leiomyosarcoma, or epithelioid sarcoma, treatment may be one of the following:

1. Surgery to remove all of the cancer. Sometimes a second operation must be done to be sure that all the tumour has been removed.
2. Surgery and radiation therapy (if the tumour cannot be completely removed by surgery).
3. A clinical trial of surgery followed by chemotherapy, for synovial sarcoma.

The effectiveness of implanted radiation therapy, radiation given during surgery, and chemotherapy given after surgery are being studied.

If your child has alveolar soft part sarcoma, treatment may be one of the following:

1. Surgery to remove all of the cancer.
2. Surgery followed by radiation therapy (if the cancer is not completely removed during surgery).

If your child has desmoplastic small round cell tumour, treatment will probably be surgery to remove as much of the tumour as possible, followed by chemotherapy and radiation therapy.

If your child has clear cell sarcoma, treatment will probably include surgery followed by radiation therapy.

If your infant has hemangioendothelioma, treatment may be one of the following:

1. Watchful waiting.
2. Surgery for tumours with symptoms.
3. Liver transplant.

If your child has a vascular tumour, treatment will probably be surgery.

Metastatic Childhood Soft Tissue Sarcoma

Children who have metastatic soft tissue sarcoma may receive combined chemotherapy, radiation therapy, and surgery to remove the cancer that has spread to the lungs.

Clinical trials are evaluating the effectiveness of combination chemotherapy and colony-stimulating factors in patients who have not undergone surgery or who have metastatic soft tissue sarcoma.

Recurrent Childhood Soft Tissue Sarcoma

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:

1. Surgery to remove the cancer.
2. Surgery followed by radiation therapy (if the patient has not yet received radiation therapy).
3. Amputation of sarcomas of the arm or leg in patients who have previously received radiation therapy.

Clinical trials are evaluating chemotherapy and biologic therapy in recurrent soft tissue sarcoma.

Childhood Soft Tissue Sarcoma

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