Soft Tissue Sarcoma (Childhood)


General Information

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.

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 Childhood Rhabdomyosarcoma Treatment for more information.) (Refer to Adult Soft Tissue Sarcoma Treatment for more information.) The soft tissue sarcomas that effect 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).

Soft tissue sarcomas are classified according to the type of soft tissue they resemble.

The types of soft tissue sarcoma include:

Tumors of fibrous (connective) tissue:
-desmoid tumor
-fibrosarcoma

Fibrohistiocytic (connective/blood tissue) tumors:
-malignant fibrous histiocytoma

Fat tissue tumors:
-liposarcoma

Smooth muscle tumors:
-leiomyosarcoma

Blood and lymph vessel tumors:
-angiosarcoma
-hemangiopericytoma
-hemangioendothelioma

Synovial (joint) tissue sarcoma:
-synovial sarcoma

Peripheral nervous system tumors:
-malignant schwannoma (malignant peripheral nerve sheath tumor)

Bone and cartilage tumors:
-extraskeletal osteosarcoma
-extraskeletal myxoid chondrosarcoma
-extraskeletal mesenchymal chondrosarcoma

Combination tissue type tumors:
-malignant mesenchymoma

Tumors of unknown origin:
-alveolar soft part sarcoma
-epitheloid sarcoma
-clear cell sarcoma (malignant melanoma of soft parts [MMSP])

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

Stage Information

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

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.

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 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 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.

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 more side effects than are desired. For these reasons, clinical trials are designed to test new treatments and to find better ways to treat cancer patients.

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 tumor 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 tumor has been removed, or if the tumor comes back following treatment.
2. Surgery to remove the cancer followed by radiation therapy (if a second surgery is not possble).
3. Chemotherapy to reduce the tumor size, followed by surgery.

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

If your child has desmoid tumor, 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, antiestrogens, and chemotherapy (if complete removal of the tumor is not possble).
3. Patients may undergo close monitoring when no other treatment alternatives are available and the tumor does not place any vital organs in danger (if complete removal of the tumor is not possible or if the tumor comes back following treatment).

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 epitheloid 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 tumor has been removed.
2. Surgery and radiation therapy (if the tumor cannot be completely removed by surgery).

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).

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.






The information on this page was obtained from the National Cancer Institute. The National Cancer Institute provides accurate, up-to-date information on many types of cancer, information on clinical trials, resources for people dealing with cancer, and information for researchers and health professionals.

The National Cancer Institute is in no way affiliated with the Mary Stolfa Cancer Foundation.

The information on this web site is provided for general information only. It is not intended as medical advice, and should not be relied upon as a substitute for consultations with qualified health professionals who are familiar with your individual medical needs. The MSCF disclaims all obligations and liabilities for damages arising from the use or attempted use of the information, including but not limited to direct, indirect, special, and consequential damages, attorneys' and experts' fees and court costs. Any use of the information will be at the risk of the user.





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