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Table of Contents General Information About Childhood Soft Tissue Sarcoma
Stages of Childhood Soft Tissue Sarcoma
Recurrent and Progressive Childhood Soft Tissue Sarcoma
Treatment Option Overview
Treatment Options for Childhood Soft Tissue Sarcoma
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Changes to This Summary (02/07/2008)
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General Information About Childhood Soft Tissue Sarcoma
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Childhood soft tissue sarcoma is a disease in which malignant (cancer) cells form in
soft tissues of the body.
Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissues include the following:
Soft tissue sarcoma may be found anywhere in the body. In children, the tumors form most often in the arms, legs, or trunk (chest and abdomen).
There are many different types of soft tissue sarcomas.
The cells of each type of sarcoma look different under a microscope. The soft tissue tumors are grouped based on the type of soft tissue cell where they first formed.
Rhabdomyosarcoma is the most common type of childhood soft tissue sarcoma. It begins in muscles that surround bone. Rhabdomyosarcoma is not discussed in this summary. (See the PDQ summary on Childhood Rhabdomyosarcoma Treatment for more information.) This summary is about the other types of soft tissue sarcoma:
Fibrous (connective) tissue tumors
Fibrohistiocytic tumors
Fat tissue tumors
Smooth muscle tumors
Blood and lymph vessel tumors
Peripheral nervous system tumors
Bone and cartilage tumors
Tumors with more than one type of tissue
Tumors of unknown origin (the place where the tumor first formed is not known)
Besides rhabdomyosarcomas, the most common soft tissue sarcomas in children are in joint tissue, connective tissue, and nerve tissue.
Soft tissue sarcoma occurs in children and adults. Soft tissue sarcoma in children may respond differently to treatment, and may have a better outcome than soft tissue sarcoma in adults. (See the PDQ summary on Adult Soft Tissue Sarcoma Treatment for more information on treatment in adults.)
Having certain diseases and inherited disorders can increase the risk of developing childhood soft tissue sarcoma.
Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors for childhood soft tissue sarcoma include having the following inherited disorders:
Other risk factors include the following:
The most common sign of childhood soft tissue sarcoma is a painless lump or swelling in soft tissues of the body.
A sarcoma may appear as a painless lump under the skin, often on an arm, a leg, or the trunk. There may be no other symptoms at first. As the sarcoma grows larger and presses on nearby organs, nerves, muscles, or blood vessels, symptoms may occur, including pain or weakness.
Other conditions may cause the same symptoms that soft tissue sarcomas do. A doctor should be consulted if any of these problems occur.
Diagnostic tests and a biopsy are used to detect (find) and diagnose childhood soft tissue sarcoma.
The following tests and procedures may be used:
- Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
- X-rays: An x-ray is a type of energy beam that can go through the body onto film, making pictures of areas inside the body. A series of x-rays may be done to check the lump or painful area.
- MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).
| Enlarge | | | | MRI of the abdomen. Patient lies on a table that slides into the MRI machine, which takes pictures of the inside of the body. The pad on the patient’s abdomen helps make the pictures clearer. |
If these tests show there may be a soft tissue sarcoma, a biopsy is done. One of the following types of biopsies may be used:
Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have a tumor. A pathologist views the tissue under a microscope to look for cancer cells and to
find out the type and grade of the tumor. The grade of a tumor depends on how abnormal the cancer cells look under a microscope and how quickly the cells are dividing. High-grade tumors usually grow and spread more quickly than low-grade tumors. Because soft tissue sarcoma can be hard to diagnose, patients should ask to have biopsy samples checked by a pathologist who has experience in diagnosing soft tissue sarcoma.
One or more of the following laboratory tests may be done to study the tissue samples:
- Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
- Immunohistochemistry study: A laboratory test in which dyes or enzymes are added to a blood or bone marrow sample to test for certain antigens (proteins that stimulate the body's immune response).
- Immunocytochemistry study: A laboratory test that uses different substances to stain (color) cells in a sample of tissue. This is used to tell the difference between the different types of soft tissue sarcoma.
- Light and electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
- The type of soft tissue sarcoma.
- The stage of the cancer (the amount of tumor remaining after surgery to remove it or whether the tumor has spread to other places in the body).
- The location, grade, and size of the tumor and how deep under the skin the tumor is.
- Whether or not the patient also has a condition called neurofibromatosis type 1 (NF1).
- The age of the patient.
- Whether the cancer has just been diagnosed or has recurred (come back).
 Back to Top Stages of Childhood Soft Tissue Sarcoma
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After childhood soft tissue sarcoma has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.
The process used to find out if cancer has spread within the soft tissue or to other
parts of the body is called staging. There is no standard staging system for childhood soft tissue sarcoma. Two methods that are commonly used for staging are based on the amount of tumor remaining after surgery to remove the tumor and/or the grade and size of the tumor and whether it has spread to the lymph nodes or other parts of the body. It is important to know
the stage in order to plan treatment.
The following tests and procedures may be used in the staging process:
- Sentinel lymph node biopsy may be used to stage childhood soft tissue sarcoma. This is the removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node to receive lymphatic drainage from a tumor. It is the first lymph node the cancer is likely to spread to from the tumor. A radioactive substance and/or blue dye is injected near the tumor. The substance or dye flows through the lymph ducts to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist views the tissue under a microscope to look for cancer cells. If cancer cells are not found, it may not be necessary to remove more lymph nodes.
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
The results of the sentinel lymph node biopsy and CT scan are viewed together with the results of
the diagnostic tests and initial surgery to determine the stage of the soft tissue sarcoma.
One method used to stage childhood soft tissue sarcoma is based on how much cancer remains after surgery to remove the tumor and whether the cancer has spread:
Nonmetastatic childhood soft tissue sarcoma
In nonmetastatic childhood soft tissue sarcoma, the cancer has been partly or completely removed by surgery and has not spread to other parts of the body.
- Group I: The tumor has been completely removed by surgery.
- Group II: After surgery to remove the tumor, there are remaining cancer cells that can be seen only with a microscope.
- Group III: After surgery, there is tumor remaining that can be seen with the eye.
Metastatic childhood soft tissue sarcoma
- Group IV: The cancer has spread from where it started to other parts of the body (metastasis).
Another method used to stage childhood soft tissue sarcoma is based on the size of the tumor and whether cancer has spread to lymph nodes or other parts of the body.
This staging system is based on the following:
- The size of the tumor.
- Whether the tumor has spread to the lymph nodes.
- Whether the tumor has spread to other parts of the body.
Back to Top Recurrent and Progressive Childhood Soft Tissue Sarcoma
Recurrent childhood soft tissue sarcoma is cancer that has recurred (come back) after it has been treated. The cancer may come back in the same place or in other parts of the body. Progressive childhood soft tissue sarcoma is cancer that did not respond to treatment.
Back to Top Treatment Option Overview
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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. Before starting treatment, patients may want to think about taking part in a clinical trial. 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.
Clinical trials are taking place in many parts of the country. Information about ongoing clinical trials is available from the NCI Web site. Choosing the most appropriate cancer treatment is a decision that ideally involves the patient, family, and health care team.
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:
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.)
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. These include the following:
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.
This summary section refers to specific treatments under study in clinical trials, but it may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.
Back to Top Treatment Options for Childhood Soft Tissue Sarcoma
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:
For treatment of desmoid tumor
Treatment of desmoid tumor will be surgery whenever possible.
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:
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:
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:
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