Childhood Soft Tissue Sarcoma
[Back to top]
Description
[Back to top]
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 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).
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:
-
Fibrohistiocytic tumors:
-
Fat tissue tumors:
-
Smooth muscle tumors:
-
Blood and lymph vessel tumors:
-
Peripheral nervous system tumors:
- malignant schwannoma (malignant peripheral nerve sheath tumor)
-
Bone and cartilage tumors:
-
Tumors with more than one type of tissue:
- malignant mesenchymoma
- malignant Triton tumor
- malignant ectomesenchymoma
-
Tumors of unknown origin:
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.
[Back to top]
Stage Explanation
[Back to top]
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.
[Back to top]
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.
[Back to top]
Metastatic childhood soft tissue sarcoma
The cancer has spread from where it started to other parts of the body.
[Back to top]
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.
[Back to top]
Treatment Option Overview
[Back to top]
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 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 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.
[Back to top]
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.
[Back to top]
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:
-
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.
- Surgery to remove the cancer followed by radiation therapy (if a second
surgery is not possible).
-
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:
- Surgery to remove all of the cancer.
- Radiation therapy given before or after surgery, nonsteroidal
antiinflammatory agents, antiestrogens, and chemotherapy (if complete
removal of the tumor is not possible).
- 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).
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:
- Surgery to remove all of the cancer. Sometimes a second operation must be
done to be sure that all the tumor has been removed.
- Surgery and radiation therapy (if the tumor cannot be completely removed
by surgery).
- 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:
- Surgery to remove all of the cancer.
- Surgery followed by radiation therapy (if the cancer is not completely
removed during surgery).
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 clear cell sarcoma, treatment will probably include surgery followed by radiation therapy.
If your infant has hemangioendothelioma, treatment may be one of the following:
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.
[Back to top]
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.
[Back to top]
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:
-
Surgery to remove the cancer.
- Surgery followed by radiation therapy (if the patient has not yet received
radiation therapy).
-
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.
[Back to top]
Changes to This Summary (10/18/2006)
The PDQ cancer information summaries are reviewed regularly and updated as
new information becomes available. This section describes the latest
changes made to this summary as of the date above.
Editorial changes were made to this summary.
This information is provided from the PDQ® database in collaboration with the NCI.
