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Soft Tissue Sarcoma

Childhood soft tissue sarcoma is a disease is which cancer (malignant) cells begin growing in soft tissue somewhere in the body. The soft tissues include muscles, tendons (bands of fiber that connect muscles to bones), fibrous 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 infants and children. If a patient has symptoms of a soft tissue sarcoma (a palpable mass), your doctor may order x-rays, other tests or refer you to a pediatric oncologist. The oncologist will refer you to a pediatric oncologic surgeon if they are concerned that the mass may be a soft tissue sarcoma. The surgeon 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.

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 (skeletal muscles) and can be found anywhere in the body. Other soft tissue sarcomas found in childhood can begin in smooth muscle, connective tissue, blood vessels, lymphatic vessels or nerve cells (nervous system).

Soft tissue sarcomas can develop anywhere in the body, but are most common in the trunk, arms and legs. The first symptom is usually a mass/lump; there is rarely fever, weight loss or night sweats. The following are common types of soft tissue sarcoma seen in children:

  • fibrosarcoma: begins in fibrous tissue in the arms and legs.
  • neurofibrosarcoma: begins in nerves near the surface of arms, legs, and trunk
  • leiomyosarcoma: begins in muscles in the trunk
  • liposarcoma: begins in fat in the arms and legs
  • synovial sarcoma: begins in linings of joint cavities and tendon sheaths of the bursa (small pockets of fluid that surround and protect the tendons) in the legs, arms and trunk.
  • hemangiopericytoma: begins in blood vessels in the arms, legs, trunk, head, and neck.
  • alveolar soft part sarcoma: begins in nerves to the muscles in the arms and legs.
  • malignant fibrous histiocytoma: begins in fibrous tissue in the legs
  • desmoid tumor (fibromatosis): is not cancer, but may be confused with a type of fibrosarcoma.

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

Stages of childhood soft tissue sarcoma

Once childhood soft tissue sarcoma 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 the stage of the cancer to plan treatment.

There are several staging systems for childhood soft tissue sarcoma, but not one that applies to all types of this form of cancer. Clinical staging is important in predicting outcome and in determining effective treatment. The treatment options outlined in this statement are based on whether the cancer has spread or the amount of tumor left after surgery.

Nonmetastatic childhood soft tissue sarcoma

The cancer is found only in the area where it started 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.

How childhood soft tissue sarcoma is treated

There are treatments for patients with childhood soft tissue sarcoma. Three types of treatment are used, most often in combination with each other:

  • 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 a common treatment for soft tissue sarcoma. Every attempt should be made to completely remove the primary tumor. However, depending on where the cancer is, the surgeon will take out as much of the cancer as possible, along with some of the normal tissue around it (negative surgical margins).

Radiation therapy uses high energy x-rays to kill cancer cells and shrink tumors. Radiation may be given before or after surgery, depending on whether tumor was left behind. 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).

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. However, the role of chemotherapy in many childhood soft tissue sarcomas remains controversial, as its effectiveness remains unclear.

Treatment by stage and clinical trial

Treatment for soft tissue sarcoma depends on where the cancer is located, how far it has spread, and what the cancer cells look like under the microscope.

The patient may receive treatment that is considered standard based on its effectiveness in patients in past clinical trials, or the doctor may recommend the patient enter a clinical trial. Clinical trials are designed to test potentially better therapy with therapy currently accepted as standard of care in hope of finding better ways to treatment cancer patients. A large cooperative group clinical trial comparing new treatments with standard treatments is going on in most parts of the country for all stages of soft tissue sarcoma. 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.

Nonmetastatic childhood soft tissue sarcoma

Treatment depends on the type of soft tissue sarcoma.

If your child has fibrosarcoma (older child or teenager), neurofibrosarcoma, liposarcoma, synovial sarcoma, hemangiopericytoma (older than 1 year of age), malignant fibrous histiocytoma, or leiomyosarcoma, 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 tumor has been removed.
  2. Surgery to remove the cancer followed by radiation therapy (if small amounts of tumor remain after surgery).
  3. Surgery to remove the cancer followed by radiation therapy and chemotherapy (if larger amounts of tumor remain after surgery).

Clinical trials are evaluating new ways to combine chemotherapy and radiation therapy with surgery as well as the effectiveness of these ways.

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

  1. Surgery to remove all the cancer. A second surgery may be needed to be certain all the tumor has been removed or if the tumor recurs.
  2. Surgery to remove all the cancer followed by radiation therapy, if a second surgery is not possible or if tumor remains.
  3. Chemotherapy to reduce tumor size, followed by surgery.

If your child has a desmoid tumor, treatment may be one the following:

  1. Surgery to remove all the cancer.
  2. Radiation therapy given before or after surgery, antiestrogens and chemotherapy, if complete removal of the tumor was not possible.
  3. Close monitoring, if complete tumor removal is not possible, if the tumor comes back or if no other alternative treatment is available and the tumor does not place vital organs in jeopardy

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

  1. Surgery to remove all the cancer.
  2. Surgery followed by radiation therapy, if not all the cancer is completely removed.
Metastatic childhood soft tissue sarcoma

Metastatic soft tissue sarcomas are treated with a combination of chemotherapy, radiation therapy and surgery to remove metastatic lung disease.

Desmoplastic round cell tumors are rare soft tissue abdominal tumors that spread rapidly and are usually metastatic at presentation. They usually occur in males in their teens and twenties. Treatment will probably be surgery, radiation, and chemotherapy.

Clinical trials are evaluating chemotherapy new combinations of chemotherapy for all metastatic soft tissue sarcomas.

Recurrent childhood soft tissue sarcoma

Treatment for recurrent childhood soft tissue sarcoma depends on what treatment your child received before, the port 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
  3. Amputation of the extremity if patient previously received radiation therapy. Clinical trials are evaluating the effectiveness of chemotherapy

Both metastatic and recurrent childhood soft tissue sarcomas are unfortunately very difficult to cure.

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; Childrens Oncology Group

© Copyright 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: 01/2003


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