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Childhood Soft Tissue Sarcoma Treatment (PDQ®)

Patient Version
Last Modified: 02/23/2012

General Information About Childhood Soft Tissue Sarcoma

Key Points for This Section


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 1 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

Peripheral nervous system tumors

Bone and cartilage tumors

PEComas

Tumors with more than one type of tissue

Tumors of unknown origin (the place where the tumor first formed is not known)

Blood and lymph vessel tumors

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 3 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 4
    Magnetic resonance imaging (MRI) of the abdomen; drawing shows the patient 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. 4
    Magnetic resonance imaging (MRI) of the abdomen. The 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:

  • Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluid using a thin needle. A pathologist views the tissue or fluid under a microscope to look for cancer cells.
  • Core biopsy: The removal of tissue using a wide needle. This procedure may be guided using ultrasound, CT scan, or MRI. A pathologist views the tissue under a microscope to look for cancer cells.
  • Incisional biopsy: The removal of part of a lump or a sample of tissue. A pathologist views the tissue under a microscope to look for cancer cells.
  • Excisional biopsy: The removal of an entire lump or area of tissue that doesn’t look normal. A pathologist views the tissue under a microscope to look for cancer cells. An excisional biopsy may be used to completely remove smaller tumors that are near the surface of the skin.

In order to plan the best treatment, a large sample of tissue may be removed during the biopsy to find out the type of soft tissue sarcoma and do laboratory tests. 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 and mid-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 the tissue sample 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 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).

Stages of Childhood Soft Tissue Sarcoma

Key Points for This Section


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.

There are three ways that cancer spreads in the body.

The three ways that cancer spreads in the body are:

  • Through tissue. Cancer invades the surrounding normal tissue.
  • Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
  • Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.

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.
Enlarge 5
Tumor size compared to everyday objects; shows various measurements of a tumor compared to a pea, peanut, walnut, and lime 5
Pea, peanut, walnut, and lime show tumor sizes.

Stage I

Stage I is divided into stages IA and IB:

Stage II

Stage II is divided into stage IIA and stage IIB:

Stage III

In stage III, the tumor is either:

Stage IV

In stage IV, the tumor is any grade, any size, and may have spread to nearby lymph nodes. Cancer has spread to distant parts of the body such as the lungs.

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.

Treatment Option Overview

Key Points for This Section


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. 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.

Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.

Children with childhood soft tissue sarcoma should have their treatment planned by a team of health care providers who are experts 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 health care providers who are experts in treating children with soft tissue sarcoma 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.

Side effects from cancer treatment that begin during or after treatment and continue for months or years 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 effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer 6 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.

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 lower the risk that the cancer will come back, 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. Stereotactic radiation therapy aims radiation directly to a tumor, causing less damage to normal tissue around the tumor. The total dose of radiation is divided into several smaller doses given over several days. This procedure is also called stereotactic external-beam radiation therapy and stereotaxic radiation therapy.

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 cerebrospinal fluid, 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.

See Drugs Approved for Adult and Childhood Sarcoma 7 for more information.

Hormone therapy

Hormone therapy is a cancer treatment that removes hormones or blocks their action and stops cancer cells from growing. Hormones are substances made 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 is 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.

This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site 8.

Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to find and attack specific cancer cells without harming normal cells. Imatinib (Gleevec) is a 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.

Other targeted therapies being studied in clinical trials include angiogenesis inhibitors. In cancer treatment, angiogenesis inhibitors prevent the growth of new blood vessels needed for tumors to grow.

Patients may want to think about taking part in a clinical trial.

For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.

Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.

Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.

Patients can enter clinical trials before, during, or after starting their cancer treatment.

Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.

Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.

Follow-up tests may be needed.

Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.

Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.

Treatment Options for Childhood Soft Tissue Sarcoma

A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.

Nonmetastatic Childhood Soft Tissue Sarcoma

For treatment of hemangiopericytoma (in infants and young children) and infantile fibrosarcoma

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:

  • Chemotherapy followed by radiation therapy, surgery, or both.
  • Radiation therapy or chemotherapy after surgery.
  • A clinical trial of internal radiation therapy.
  • A clinical trial of radiation therapy given during surgery.
  • A clinical trial of surgery followed by watchful waiting, chemotherapy, and/or radiation therapy, based on the grade of the tumor and the amount of tumor that is removed by surgery. Sometimes chemotherapy may be given before surgery.

For treatment of desmoid tumor

Treatment of desmoid tumor will be surgery whenever possible. Other treatments may include watchful waiting, chemotherapy, or surgery followed by internal radiation therapy.

If surgery to completely remove the tumor is not possible, treatment to shrink the tumor before surgery may include the following:

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 clear cell sarcoma

Treatment of clear cell 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 may include surgery, chemotherapy (which may be given before surgery), and radiation therapy.

For treatment of extraosseous osteosarcoma

See the PDQ summary on Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment 2 for more information on the treatment of extraosseous osteosarcoma.

For treatment of malignant peripheral nerve sheath tumor

Treatment of malignant peripheral nerve sheath tumor will be surgery whenever possible. Other treatments may include the following:

For treatment of plexiform histiocytic tumor

Treatment of plexiform histiocytic tumor is surgery to completely remove the tumor.

For treatment of synovial sarcoma

Treatment of synovial sarcoma may include the following:

For treatment of undifferentiated soft tissue sarcoma

Treatment of undifferentiated soft tissue sarcoma may be within a clinical trial for patients with nonrhabdomyosarcomatous soft tissue sarcomas (NRSTS). This study includes surgery and/or radiation therapy with or without chemotherapy.

For treatment of angiosarcoma and lymphangiosarcoma

Treatment of angiosarcoma and lymphangiosarcoma may include the following:

For treatment of hemangioendothelioma

Treatment of hemangioendothelioma in children younger than one year may include the following:

Treatment of hemangioendothelioma in children aged one year and older may include the following:

For treatment of aggressive fibromatosis, dermatofibrosarcoma, and angiomatoid malignant fibrous histiocytoma

Treatment of these tumor types will be surgery whenever possible. Other treatments may include the following:

For treatment of epithelioid sarcoma, leiomyosarcoma, liposarcoma, and mesenchymal chondrosarcoma

Treatment of these tumor types will be surgery whenever possible. Other treatments may include the following:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with nonmetastatic childhood soft tissue sarcoma 9. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 10.

Metastatic Childhood Soft Tissue Sarcoma

Treatment of metastatic childhood soft tissue sarcoma may include the following:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with metastatic childhood soft tissue sarcoma 11. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 10.

Recurrent and Progressive Childhood Soft Tissue Sarcoma

Treatment of recurrent or progressive childhood soft tissue sarcoma may include the following:

Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent childhood soft tissue sarcoma 12. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 10.

Changes to This Summary (02/23/2012)

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.

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About PDQ

PDQ is a comprehensive cancer database available on NCI's Web site.

PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site 29. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.

PDQ contains cancer information summaries.

The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.

The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.

Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.

PDQ also contains information on clinical trials.

A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness.

Listings of clinical trials are included in PDQ and are available online at NCI's Web site 10. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).

The PDQ database contains listings of groups specializing in clinical trials.

The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site 29 or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).



Glossary Terms

abdomen (AB-doh-men)
The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.
abnormal (ab-NOR-mul)
Not normal. An abnormal lesion or growth may be cancer, premalignant (likely to become cancer), or benign (not cancer).
AIDS
A disease caused by the human immunodeficiency virus (HIV). People with AIDS are at an increased risk for developing certain cancers and for infections that usually occur only in individuals with a weak immune system. Also called acquired immunodeficiency syndrome.
alveolar soft part sarcoma (al-VEE-oh-ler … sar-KOH-muh)
A soft tissue tumor that is most common in older children and teenagers. It begins in the soft supporting tissue that connects and surrounds the organs and other tissues. Alveolar soft part sarcoma usually occurs in the legs, but can also occur in the arms, hands, head, or neck. It can cause the growth of new blood vessels that help the tumor grow and spread. Also called ASPS.
angiomyolipoma (AN-jee-oh-MY-oh-lih-POH-muh)
A benign (noncancer) tumor of fat and muscle tissue that usually is found in the kidney. Angiomyolipomas rarely cause symptoms, but may bleed or grow large enough to be painful or cause kidney failure. They are common in patients with tuberous sclerosis (a genetic disorder in which benign tumors grow in the kidneys, brain, eyes, heart, lungs, and skin, causing seizures, mental problems, and skin lesions).
angiosarcoma (AN-jee-oh-sar-KOH-muh)
A type of cancer that begins in the cells that line blood vessels or lymph vessels. Cancer that begins in blood vessels is called hemangiosarcoma. Cancer that begins in lymph vessels is called lymphangiosarcoma.
antigen (AN-tih-jen)
Any substance that causes the body to make a specific immune response.
biopsy (BY-op-see)
The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.
blood (blud)
A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.
blood vessel (blud VEH-sel)
A tube through which the blood circulates in the body. Blood vessels include a network of arteries, arterioles, capillaries, venules, and veins.
bone marrow (bone MAYR-oh)
The soft, sponge-like tissue in the center of most bones. It produces white blood cells, red blood cells, and platelets.
cancer (KAN-ser)
A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord. Also called malignancy.
cell (sel)
The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
chondrosarcoma (KON-droh-sar-KOH-muh)
A type of cancer that forms in bone cartilage. It usually starts in the pelvis (between the hip bones), the shoulder, the ribs, or at the ends of the long bones of the arms and legs. A rare type of chondrosarcoma called extraskeletal chondrosarcoma does not form in bone cartilage. Instead, it forms in the soft tissues of the upper part of the arms and legs. Chondrosarcoma can occur at any age but is more common in people older than 40 years. It is a type of bone cancer.
chromosome (KROH-muh-some)
Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.
clear cell (kleer sel)
A type of cell that looks clear inside when viewed under a microscope.
clear cell sarcoma of soft tissue (kleer sel sar-KOH-muh ... TIH-shoo)
A soft tissue tumor that begins in a tendon (tough, fibrous, cord-like tissue that connects muscle to bone or to another structure). Clear cell sarcoma of soft tissue has certain markers that are also found on malignant melanoma (a type of skin cancer). It usually occurs in the leg or arm. Also called malignant melanoma of soft parts.
clinical trial (KLIH-nih-kul TRY-ul)
A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called clinical study.
condition (kun-DIH-shun)
In medicine, a health problem with certain characteristics or symptoms.
connective tissue (kuh-NEK-tiv TIH-shoo)
Supporting tissue that surrounds other tissues and organs. Specialized connective tissue includes bone, cartilage, blood, and fat.
core biopsy (... BY-op-see)
The removal of a tissue sample with a wide needle for examination under a microscope. Also called core needle biopsy.
CT scan (… skan)
A series of detailed pictures of areas inside the body taken from different angles. The pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.
cytogenetics (SY-toh-jeh-NEH-tix)
The study of chromosomes and chromosomal abnormalities.
dermatofibrosarcoma protuberans (DER-muh-toh-FY-broh-sar-KOH-muh proh-TOO-beh-ranz)
A type of tumor that begins as a hard nodule and grows slowly. These tumors are usually found in the dermis (the inner layer of the two main layers of tissue that make up the skin) of the limbs or trunk of the body. They can grow into surrounding tissue but do not spread to other parts of the body. These tumors are related to giant cell fibroblastomas.
desmoid tumor (DEZ-moyd TOO-mer)
A tumor of the tissue that surrounds muscles, usually in the abdomen. A desmoid tumor rarely metastasizes (spreads to other parts of the body). It may be called aggressive fibromatosis when the tumor is outside of the abdomen.
desmoplastic small round cell tumor (DES-moh-PLAS-tik ... TOO-mer)
A rare, aggressive cancer that usually affects young males and usually is located in the abdomen.
diagnosis (DY-ug-NOH-sis)
The process of identifying a disease, such as cancer, from its signs and symptoms.
disorder (dis-OR-der)
In medicine, a disturbance of normal functioning of the mind or body. Disorders may be caused by genetic factors, disease, or trauma.
electron microscope (ee-LEK-tron MY-kroh-SKOPE)
A microscope (device used to magnify small objects) that uses electrons (instead of light) to produce an enlarged image. An electron microscope shows tiny details better than any other type of microscope.
enzyme (EN-zime)
A protein that speeds up chemical reactions in the body.
Epstein-Barr virus (ep-stine-BAR VY-rus)
A common virus that remains dormant in most people. It causes infectious mononucleosis and has been associated with certain cancers, including Burkitt lymphoma, immunoblastic lymphoma, and nasopharyngeal carcinoma. Also called EBV.
excisional biopsy (ek-SIH-zhuh-nul BY-op-see)
A surgical procedure in which an entire lump or suspicious area is removed for diagnosis. The tissue is then examined under a microscope.
extraosseous (EK-struh-AH-see-us)
Located outside of the bone.
familial adenomatous polyposis (fuh-MIH-lee-ul A-deh-NOH-muh-tus PAH-lee-POH-sis)
An inherited condition in which numerous polyps (growths that protrude from mucous membranes) form on the inside walls of the colon and rectum. It increases the risk of colorectal cancer. Also called familial polyposis and FAP.
fibrosarcoma (FY-broh-sar-KOH-muh)
A type of soft tissue sarcoma that begins in fibrous tissue, which holds bones, muscles, and other organs in place.
fine-needle aspiration biopsy (... NEE-dul AS-pih-RAY-shun BY-op-see)
The removal of tissue or fluid with a thin needle for examination under a microscope. Also called FNA biopsy.
fluid (FLOO-id)
A substance that flows smoothly and takes the shape of its container. Liquids and gases are fluids.
grade (grayd)
A description of a tumor based on how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. Grading systems are different for each type of cancer.
hemangiopericytoma (hee-MAN-jee-oh-PAYR-ih-sy-TOH-muh)
A type of cancer involving blood vessels and soft tissue.
high grade (hy grayd)
A term used to describe cells that look abnormal under a microscope. These cells are more likely to grow and spread quickly than cells in low-grade cancer or in growths that may become cancer.
immune response (ih-MYOON reh-SPONTS)
The activity of the immune system against foreign substances (antigens).
immunohistochemistry (IH-myoo-noh-HIS-toh-KEH-mih-stree)
A technique used to identify specific molecules in different kinds of tissue. The tissue is treated with antibodies that bind the specific molecule. These are made visible under a microscope by using a color reaction, a radioisotope, colloidal gold, or a fluorescent dye. Immunohistochemistry is used to help diagnose diseases, such as cancer, and to detect the presence of microorganisms. It is also used in basic research to understand how cells grow and differentiate (become more specialized).
incisional biopsy (in-SIH-zhuh-nul BY-op-see)
A surgical procedure in which a portion of a lump or suspicious area is removed for diagnosis. The tissue is then examined under a microscope to check for signs of disease.
infection (in-FEK-shun)
Invasion and multiplication of germs in the body. Infections can occur in any part of the body and can spread throughout the body. The germs may be bacteria, viruses, yeast, or fungi. They can cause a fever and other problems, depending on where the infection occurs. When the body’s natural defense system is strong, it can often fight the germs and prevent infection. Some cancer treatments can weaken the natural defense system.
inherited (in-HAYR-ih-ted)
Transmitted through genes that have been passed from parents to their offspring (children).
joint (joynt)
In medicine, the place where two or more bones are connected. Examples include the shoulder, elbow, knee, and jaw.
laboratory test (LA-bruh-tor-ee...)
A medical procedure that involves testing a sample of blood, urine, or other substance from the body. Tests can help determine a diagnosis, plan treatment, check to see if treatment is working, or monitor the disease over time.
leiomyosarcoma (LY-oh-MY-oh-sar-KOH-muh)
A malignant (cancer) tumor of smooth muscle cells that can arise almost anywhere in the body, but is most common in the uterus, abdomen, or pelvis.
Li-Fraumeni syndrome (lee-FRAH-meh-nee SIN-drome)
A rare, inherited predisposition to multiple cancers, caused by an alteration in the p53 tumor suppressor gene.
liposarcoma (LIH-poh-sar-KOH-muh)
A rare cancer of the fat cells.
low grade (loh grayd)
A term used to describe cells that look nearly normal under a microscope. These cells are less likely to grow and spread more quickly than cells in high-grade cancer or in growths that may become cancer.
lymph node (limf node)
A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.
lymph vessel (limf ...)
A thin tube that carries lymph (lymphatic fluid) and white blood cells through the lymphatic system. Also called lymphatic vessel.
lymphangiosarcoma (lim-FAN-jee-oh-sar-KOH-muh)
A type of cancer that begins in the cells that line lymph vessels.
malignant (muh-LIG-nunt)
Cancerous. Malignant cells can invade and destroy nearby tissue and spread to other parts of the body.
malignant ectomesenchymoma (muh-LIG-nunt EK-toh-MEH-zen-ky-MOH-muh)
A rare, fast-growing tumor of the nervous system or soft tissue that occurs in children and young adults. Malignant ectomesenchymomas may form in the head and neck, abdomen, perineum, scrotum, or limbs. Also called ectomesenchymoma.
malignant fibrous histiocytoma (muh-LIG-nunt FY-brus HIS-tee-oh-sy-TOH-muh)
A soft tissue sarcoma that usually occurs in the limbs, most commonly the legs, and may also occur in the abdomen. Also called malignant fibrous cytoma.
medical history (MEH-dih-kul HIH-stuh-ree)
A record of information about a person’s health. A personal medical history may include information about allergies, illnesses, surgeries, immunizations, and results of physical exams and tests. It may also include information about medicines taken and health habits, such as diet and exercise. A family medical history includes health information about a person’s close family members (parents, grandparents, children, brothers, and sisters). This includes their current and past illnesses. A family medical history may show a pattern of certain diseases in a family.
melanoma (MEH-luh-NOH-muh)
A form of cancer that begins in melanocytes (cells that make the pigment melanin). It may begin in a mole (skin melanoma), but can also begin in other pigmented tissues, such as in the eye or in the intestines.
mesenchymal (meh-ZEN-kih-mul)
Refers to cells that develop into connective tissue, blood vessels, and lymphatic tissue.
microscope (MY-kroh-SKOPE)
An instrument that is used to look at cells and other small objects that cannot be seen with the eye alone.
MRI
A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, NMRI, and nuclear magnetic resonance imaging.
nerve (nerv)
A bundle of fibers that receives and sends messages between the body and the brain. The messages are sent by chemical and electrical changes in the cells that make up the nerves.
neurofibromatosis type 1 (NOOR-oh-FY-broh-muh-TOH-sis ...)
A rare genetic condition that causes brown spots and tumors on the skin, freckling in skin areas not exposed to the sun, tumors on the nerves, and developmental changes in the nervous system, muscles, bone, and skin. Also called NF1.
organ (OR-gun)
A part of the body that performs a specific function. For example, the heart is an organ.
osteosarcoma (OS-tee-oh-sar-KOH-muh)
A cancer of the bone that usually affects the large bones of the arm or leg. It occurs most commonly in young people and affects more males than females. Also called osteogenic sarcoma.
outcome (OWT-kum)
A specific result or effect that can be measured. Examples of outcomes include decreased pain, reduced tumor size, and improvement of disease.
pathologist (puh-THAH-loh-jist)
A doctor who identifies diseases by studying cells and tissues under a microscope.
PDQ
PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.
physical examination (FIH-zih-kul eg-ZA-mih-NAY-shun)
An exam of the body to check for general signs of disease.
pleomorphic (PLEE-oh-MOR-fik)
Occurring in various distinct forms. In terms of cells, having variation in the size and shape of cells or their nuclei.
plexiform fibrohistiocytic tumor (PLEK-sih-form FY-broh-HIS-tee-oh-SIH-tik TOO-mer)
A rare tumor found mainly in children and young adults. It usually forms in the skin on the arms and legs. It is slow-growing and usually does not spread to other parts of the body. It is a type of soft tissue tumor.
primary tumor (PRY-mayr-ee TOO-mer)
The original tumor.
prognosis (prog-NO-sis)
The likely outcome or course of a disease; the chance of recovery or recurrence.
protein (PROH-teen)
A molecule made up of amino acids that are needed for the body to function properly. Proteins are the basis of body structures such as skin and hair and of substances such as enzymes, cytokines, and antibodies.
radiation therapy (RAY-dee-AY-shun THAYR-uh-pee)
The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called irradiation and radiotherapy.
recover (ree-KUH-ver)
To become well and healthy again.
recur (ree-KER)
To come back or to return.
recurrent cancer (ree-KER-ent KAN-ser)
Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrence.
response (reh-SPONTS)
In medicine, an improvement related to treatment.
retinoblastoma (REH-tih-noh-blas-TOH-muh)
Cancer that forms in the tissues of the retina (the light-sensitive layers of nerve tissue at the back of the eye). Retinoblastoma usually occurs in children younger than 5 years. It may be hereditary or nonhereditary (sporadic).
rhabdomyosarcoma (RAB-doh-MY-oh-sar-KOH-muh)
Cancer that forms in the soft tissues in a type of muscle called striated muscle. Rhabdomyosarcoma can occur anywhere in the body.
risk factor (... FAK-ter)
Something that increases the chance of developing a disease. Some examples of risk factors for cancer are age, a family history of certain cancers, use of tobacco products, being exposed to radiation or certain chemicals, infection with certain viruses or bacteria, and certain genetic changes.
sarcoma (sar-KOH-muh)
A cancer of the bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue.
schwannoma (shwah-NOH-muh)
A tumor of the peripheral nervous system that arises in the nerve sheath (protective covering). It is almost always benign, but rare malignant schwannomas have been reported.
soft tissue (... TIH-shoo)
Refers to muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.
soft tissue sarcoma (…TIH-shoo sar-KOH-muh)
A cancer that begins in the muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.
spindle cell sarcoma (SPIN-dul sel sar-KOH-muh)
A type of connective tissue cancer in which the cells are spindle-shaped when examined under a microscope.
stage (stayj)
The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.
surgery (SER-juh-ree)
A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.
symptom (SIMP-tum)
An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.
synovial membrane (sih-NOH-vee-ul MEM-brayn)
A layer of connective tissue that lines the cavities of joints, tendon sheaths, and bursae (fluid-filled sacs between tendons and bones). The synovial membrane makes synovial fluid, which has a lubricating function.
synovial sarcoma (sih-NOH-vee-ul sar-KOH-muh)
A malignant tumor that develops in the synovial membrane of the joints.
tendon (TEN-dun)
Tough, fibrous, cord-like tissue that connects muscle to bone or another structure, such as an eyeball. Tendons help the bone or structure to move.
tissue (TIH-shoo)
A group or layer of cells that work together to perform a specific function.
tumor (TOO-mer)
An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.
ultrasound-guided biopsy (UL-truh-SOWND-GY-ded BY-op-see)
A biopsy procedure that uses an ultrasound imaging device to find an abnormal area of tissue and guide its removal for examination under a microscope.
undifferentiated (un-DIH-feh-REN-shee-AY-ted)
A term used to describe cells or tissues that do not have specialized ("mature") structures or functions. Undifferentiated cancer cells often grow and spread quickly.
x-ray (EX-ray)
A type of radiation used in the diagnosis and treatment of cancer and other diseases. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.

Table of Links

1http://www.cancer.gov/cancertopics/pdq/treatment/childrhabdomyosarcoma/Patient
2http://www.cancer.gov/cancertopics/pdq/treatment/osteosarcoma/Patient
3http://www.cancer.gov/cancertopics/pdq/treatment/adult-soft-tissue-sarcoma/Pati
ent
4http://www.cancer.gov/PublishedContent/MediaLinks/291947.html
5http://www.cancer.gov/PublishedContent/MediaLinks/291948.html
6http://www.cancer.gov/cancertopics/pdq/treatment/lateeffects/Patient
7http://www.cancer.gov/cancertopics/druginfo/soft-tissue-sarcoma#dal1
8http://cancer.gov/clinicaltrials
9http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=40257&tt=1&a
mp;format=1&cn=1
10http://www.cancer.gov/clinicaltrials
11http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=40258&tt=1&a
mp;format=1&cn=1
12http://www.cancer.gov/Search/ClinicalTrialsLink.aspx?Diagnosis=40259&tt=1&a
mp;format=1&cn=1
13http://www.cancer.gov/cancertopics/types/soft-tissue-sarcoma
14http://www.cancer.gov/cancertopics/understandingcancer/targetedtherapies
15http://www.cancer.gov/cancertopics/factsheet/Therapy/targeted
16http://www.cancer.gov/cancertopics/wyntk/overview
17http://www.cancer.gov/cancertopics/types/childhoodcancers
18http://www.curesearch.org
19http://www.cancer.gov/cancertopics/aya
20http://www.cancer.gov/cancertopics/youngpeople
21http://www.cancer.gov/cancertopics/factsheet/NCI/children-adolescents
22http://www.cancer.gov/cancertopics/understandingcancer/cancer
23http://www.cancer.gov/cancertopics/factsheet/Detection/staging
24http://www.cancer.gov/cancertopics/coping
25http://www.cancer.gov/cancertopics/cancerlibrary/questions
26http://www.cancer.gov/cancertopics/literature
27http://dccps.nci.nih.gov/ocs/resources.html
28https://livehelp.cancer.gov
29http://cancer.gov
30https://pubs.cancer.gov/ncipl