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

Nonmetastatic Childhood Soft Tissue Sarcoma Metastatic Childhood Soft Tissue Sarcoma Recurrent and Progressive Childhood Soft Tissue Sarcoma

To Learn More About Childhood Soft Tissue Sarcoma
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Changes to This Summary (10/31/2008)
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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. There are many different types of soft tissue sarcomas. 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) Having certain diseases and inherited disorders can increase the risk of developing childhood soft tissue sarcoma. The most common sign of childhood soft tissue sarcoma is a painless lump or swelling in soft tissues of the body. Diagnostic tests and a biopsy are used to detect (find) and diagnose childhood soft tissue sarcoma. Certain factors affect prognosis (chance of recovery) and treatment options.

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:

• Muscles.
• Tendons (bands of tissue that connect muscles to bones).
• Synovial tissues (tissues around joints).
• Fat.
• Blood vessels.
• Lymph vessels.
• Nerves.

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

• Fibromatoses (desmoid tumor).
• Dermatofibrosarcoma.
• Fibrosarcoma.
• Inflammatory myofibroblastic tumor.

Fibrohistiocytic tumors

• Malignant fibrous histiocytoma (also called undifferentiated pleomorphic sarcoma, or spindle cell sarcoma).

Fat tissue tumors

• Liposarcoma.

Smooth muscle tumors

• Leiomyosarcoma.

Blood and lymph vessel tumors

• Angiosarcoma.
• Lymphangiosarcoma.
• Hemangiopericytoma.
• Hemangioendothelioma.

Peripheral nervous system tumors

• Malignant schwannoma (malignant peripheral nerve sheath tumor).

Bone and cartilage tumors

• Extraosseous osteosarcoma.
• Extraosseous myxoid chondrosarcoma.
• Extraosseous mesenchymal chondrosarcoma.

Tumors with more than one type of tissue

• Malignant mesenchymoma.
• Malignant Triton tumor.
• Malignant ectomesenchymoma.

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

• Alveolar soft part sarcoma.
• Epithelioid sarcoma.
• Clear cell sarcoma (malignant melanoma of soft parts).
• Synovial sarcoma.
• Desmoplastic small round cell tumor.

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 ² 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:

• Li-Fraumeni syndrome.
• Neurofibromatosis type 1 (NF1).

Other risk factors include the following:

• Having AIDS (acquired immune deficiency syndrome) and Epstein-Barr virus infection.
• Having retinoblastoma in both eyes.
• Past treatment with radiation therapy.

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

  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.
• Core biopsy: The removal of tissue using a wide needle.
• Incisional biopsy: The removal of part of a lump or a sample of tissue.
• Excisional biopsy: The removal of an entire lump of tissue. 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 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).

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. There are three ways that cancer spreads in the body. 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 Metastatic childhood soft tissue sarcoma 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. Sometimes the stages used for adult soft tissue sarcoma are used for childhood soft tissue sarcoma: Stage I Stage II Stage III Stage IV

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.

Sometimes the stages used for adult soft tissue sarcoma are used for childhood soft tissue sarcoma:

Stage I

In stage I, the tumor is any size, low-grade (likely to grow and spread slowly), and may be either superficial (close to the skin's surface) or deep.

Enlarge

Pea, peanut, walnut, and lime show tumor sizes.

Stage II

In stage II, the tumor is high-grade (likely to grow and spread quickly) and either:

• 5 centimeters or smaller and either superficial (close to the skin's surface) or deep; or
• larger than 5 centimeters and superficial.

Stage III

In stage III, the tumor is high-grade, larger than 5 centimeters, and deep.

Stage IV

In stage IV, the tumor is any size, any grade, and has spread to nearby lymph nodes and/or to other parts of the body.

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. 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. Some cancer treatments cause side effects months or years after treatment has ended. Seven types of standard treatment are used: Surgery Radiation therapy Chemotherapy Hormone therapy Watchful waiting Nonsteroidal anti-inflammatory drugs Liver transplant New types of treatment are being tested in clinical trials. Targeted drug therapy Patients may want to think about taking part in a clinical trial. Patients can enter clinical trials before, during, or after starting their cancer treatment. Follow-up tests may be needed.

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:

• Radiation oncologist.
• Pediatric hematologist.
• Pediatric nurse specialist.
• Rehabilitation specialist.
• Psychologist.
• Social worker.

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 ³ 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 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 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.

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 ⁴.

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.

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 clinical trials database.

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.

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:

• Chemotherapy followed by radiation therapy, surgery, or both.
• A clinical trial of internal radiation therapy.
• A clinical trial of radiation therapy given during surgery.

For treatment of desmoid tumor

Treatment of desmoid tumor will be surgery whenever possible.

• If surgery to completely remove the tumor is not possible, treatment may include the following:
  • External radiation therapy to shrink the tumor before surgery.
  • Internal radiation therapy to kill any remaining tumor cells after surgery.
  • A nonsteroidal anti-inflammatory drug (NSAID).
  • Antiestrogen therapy.
  • Chemotherapy.
• Watchful waiting.
• A clinical trial of targeted drug therapy with imatinib (Gleevec).

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:

• Chemotherapy followed by radiation therapy, surgery, or both.
• A clinical trial of internal radiation therapy.
• A clinical trial of radiation therapy given during surgery.

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:

• Radiation therapy after surgery.
• A clinical trial of surgery with or without chemotherapy and/or radiation therapy, based on the grade of the tumor and the amount of tumor that is removed by surgery..

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:

• Surgery to remove the tumor.
• Treatment for blood clotting disorders.
• Liver transplant (if the tumor is in the liver).

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

• Watchful waiting as some tumors will disappear without treatment.
• Treatment for blood clotting disorders.
• Surgery to remove the tumor if there are symptoms.

For treatment of angiosarcoma and lymphangiosarcoma

Treatment of angiosarcoma and lymphangiosarcoma may include the following:

• Surgery to completely remove the tumor.
• Radiation therapy after surgery.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with nonmetastatic childhood soft tissue sarcoma ⁵. 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 ⁶.

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

• Combination chemotherapy, radiation therapy, and surgery (thoracotomy) to remove cancer that has spread to the lungs.
• A clinical trial of combination chemotherapy and granulocyte colony-stimulating factor (substance that helps the body increase the number of white blood cells).

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with metastatic childhood soft tissue sarcoma ⁷. 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 ⁶.

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

• Surgery followed by radiation therapy, if not already given.
• Surgery to remove the arm or leg with cancer, if radiation therapy was already given.
• Surgery to remove cancer that has spread to the lungs.
• A clinical trial of chemotherapy or targeted drug therapy with imatinib (Gleevec).
• A clinical trial of a new treatment.

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent childhood soft tissue sarcoma ⁸. 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 ⁶.

To Learn More About Childhood Soft Tissue Sarcoma

For more information from the National Cancer Institute about childhood soft tissue sarcoma, see the following:

• Soft Tissue Sarcomas: Questions and Answers ⁹
• Synovial Sarcoma: Questions and Answers ¹⁰

For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:

• What You Need to Know About™ Cancer - An Overview ¹¹
• Childhood Cancers ¹²
• CureSearch - National Childhood Cancer Foundation Children’s Oncology Group ¹³
• Late Effects of Treatment for Childhood Cancer ³
• Young People with Cancer: A Handbook for Parents ¹⁴
• Care for Children and Adolescents with Cancer: Questions and Answers ¹⁵
• Understanding Cancer Series: Cancer ¹⁶
• Staging: Questions and Answers ¹⁷
• Coping with Cancer ¹⁸
• Support and Resources ¹⁹
• Cancer Library ²⁰
• Information for Survivors/Caregivers/Advocates ²¹

Get More Information From NCI

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Changes to This Summary (10/31/2008)

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.

Changes were made to this summary to match those made to the health professional version.

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 ²³. 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 ²⁵. 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), TTY at 1-800-332-8615.

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 ²³ or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

The PDQ database contains listings of cancer health professionals and hospitals with cancer programs.

Because cancer in children and adolescents is rare, the majority of children with cancer are treated by health professionals specializing in childhood cancers, at hospitals or cancer centers with special facilities to treat them. The PDQ database contains listings of health professionals who specialize in childhood cancer and listings of hospitals with cancer programs. For help locating childhood cancer health professionals or a hospital with cancer programs, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237), TTY at 1-800-332-8615.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

Not normal. An abnormal lesion or growth may be cancerous, premalignant (likely to become cancer), or benign.

Treatment given after the primary treatment to increase the chances of a cure. Adjuvant therapy may include chemotherapy, radiation therapy, hormone therapy, or biological therapy.

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.

Surgery to remove part or all of a limb or appendage.

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.

A substance that prevents cells from making or using estrogen (a hormone that plays a role in the formation of female sex characteristics, the menstrual cycle, and pregnancy). Antiestrogens may stop some cancer cells from growing and are used to prevent and treat breast cancer. They are also being studied in the treatment of other types of cancer. An antiestrogen is a type of hormone antagonist.

A substance that causes the immune system to make a specific immune response.

A drug that reduces pain, fever, inflammation, and blood clotting. Aspirin belongs to the family of drugs called nonsteroidal anti-inflammatory agents. It is also being studied in cancer prevention.

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.

Tissue removed from the body and examined under a microscope to determine whether disease is present.

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.

A mass of blood that forms when blood platelets, proteins, and cells stick together. When a blood clot is attached to the wall of a blood vessel, it is called a thrombus. When it moves through the bloodstream and blocks the flow of blood in another part of the body, it is called an embolus.

A tube through which the blood circulates in the body. Blood vessels include a network of arteries, arterioles, capillaries, venules, and veins.

Primary bone cancer is cancer that forms in cells of the bone. Some types of primary bone cancer are osteosarcoma, Ewing sarcoma, malignant fibrous histiocytoma, and chondrosarcoma. Secondary bone cancer is cancer that spreads to the bone from another part of the body (such as the prostate, breast, or lung).

The soft, sponge-like tissue in the center of most bones. It produces white blood cells, red blood cells, and platelets.

Cancer that forms in tissues of the breast, usually the ducts (tubes that carry milk to the nipple) and lobules (glands that make milk). It occurs in both men and women, although male breast cancer is rare.

A term for diseases in which abnormal cells divide without control. Cancer cells can invade nearby tissues and can spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is 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.

The smallest type of blood vessel. A capillary connects an arteriole (small artery) to a venule (small vein) to form a network of blood vessels in almost all parts of the body. The wall of a capillary is thin and leaky, and capillaries are involved in the exchange of fluids and gases between tissues and the blood.

A flexible tube used to deliver fluids into or withdraw fluids from the body.

A hollow area or hole. It may describe a body cavity (such as the space within the abdomen) or a hole in a tooth caused by decay.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

A measure of length in the metric system. There are 100 centimeters in a meter and 2½ centimeters in an inch.

Treatment with drugs that kill cancer cells.

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.

Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.

A type of cell that looks clear inside when viewed under a microscope.

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.

Treatment using more than one anticancer drug.

Supporting tissue that surrounds other tissues and organs. Specialized connective tissue includes bone, cartilage, blood, and fat.

A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.

The removal of a tissue sample with a wide needle for examination under a microscope.

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.

To heal or restore health; a treatment to restore health.

The study of chromosomes and chromosomal abnormalities.

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.

A rare, aggressive cancer that usually affects young males and usually is located in the abdomen.

The process of identifying a disease, such as cancer, from its signs and symptoms.

A method used to identify a disease.

In medicine, a disturbance of normal functioning of the mind or body. Disorders may be caused by genetic factors, disease, or trauma.

Any substance, other than food, that is used to prevent, diagnose, treat or relieve symptoms of a disease or abnormal condition. Also refers to a substance that alters mood or body function, or that can be habit-forming or addictive, especially a narcotic.

In medicine, a tube or vessel of the body through which fluids pass.

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.

A protein that speeds up chemical reactions in the body.

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.

A type of hormone made by the body that helps develop and maintain female sex characteristics and the growth of long bones. Estrogens can also be made in the laboratory. They may be used as a type of birth control and to treat symptoms of menopause, menstrual disorders, osteoporosis, and other conditions.

A surgical procedure in which an entire lump or suspicious area is removed for diagnosis. The tissue is then examined under a microscope.

A type of radiation therapy that uses a machine to aim high-energy rays at the cancer from outside of the body. Also called external-beam radiation therapy.

Located outside of the bone.

An increase in body temperature above normal (98.6 degrees F), usually caused by disease.

A condition in which multiple fibromas develop. Fibromas are tumors (usually benign) that affect connective tissue.

A type of soft tissue sarcoma that begins in fibrous tissue, which holds bones, muscles, and other organs in place.

The removal of tissue or fluid with a thin needle for examination under a microscope. Also called FNA biopsy.

Liquid.

Monitoring a person's health over time after treatment. This includes keeping track of the health of people who participate in a clinical study or clinical trial for a period of time, both during the study and after the study ends.

An organ that makes one or more substances, such as hormones, digestive juices, sweat, tears, saliva, or milk. Endocrine glands release the substances directly into the bloodstream. Exocrine glands release the substances into a duct or opening to the inside or outside of the body.

The grade of a tumor depends 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.

Healthy skin, bone, or other tissue taken from one part of the body and used to replace diseased or injured tissue removed from another part of the body.

A colony-stimulating factor that stimulates the production of neutrophils (a type of white blood cell). It is a cytokine that is a type of hematopoietic (blood-forming) agent. Also called filgrastim and G-CSF.

A type of cancer involving blood vessels and soft tissue.

When referring to cancerous and precancerous growths, 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 cancerous and precancerous growths.

One of many chemicals made by glands in the body. Hormones circulate in the bloodstream and control the actions of certain cells or organs. Some hormones can also be made in the laboratory.

Treatment that adds, blocks, or removes hormones. For certain conditions (such as diabetes or menopause), hormones are given to adjust low hormone levels. To slow or stop the growth of certain cancers (such as prostate and breast cancer), synthetic hormones or other drugs may be given to block the body’s natural hormones. Sometimes surgery is needed to remove the gland that makes a certain hormone. Also called endocrine therapy, hormonal therapy, and hormone treatment.

A drug used to treat different types of leukemia and other cancers of the blood, gastrointestinal stromal tumors, skin tumors called dermatofibrosarcoma protuberans, and a rare condition called systemic mastocytosis. It is also being studied in the treatment of other types of cancer. Imatinib mesylate blocks the protein made by the bcr/abl oncogene. It is a type of tyrosine kinase inhibitor. Also called Gleevec and STI571.

The activity of the immune system against foreign substances (antigens).

A substance or object that is put in the body as a prosthesis, or for treatment or diagnosis.

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.

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.

Having to do with inflammation (redness, swelling, pain, and a feeling of heat that helps protect tissues affected by injury or disease).

Transmitted through genes that have been passed from parents to their offspring (children).

Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."

A type of radiation therapy in which radioactive material sealed in needles, seeds, wires, or catheters is placed directly into or near a tumor. Also called brachytherapy, implant radiation therapy, and radiation brachytherapy.

Cancer that has spread beyond the layer of tissue in which it developed and is growing into surrounding, healthy tissues. Also called infiltrating cancer.

In medicine, the place where two or more bones are connected. Examples include the shoulder, elbow, knee, and jaw.

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.

Side effects of cancer treatment that appear months or years after treatment has ended. Late effects include physical and mental problems and second cancers.

A malignant (cancerous) tumor of smooth muscle cells that can arise almost anywhere in the body, but is most common in the uterus, abdomen, or pelvis.

A rare, inherited predisposition to multiple cancers, caused by an alteration in the p53 tumor suppressor gene.

A rare cancer of the fat cells.

A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.

When referring to cancerous and precancerous growths, a term used to describe cells that look nearly normal under a microscope. These cells are less likely to grow and spread quickly than cells in high-grade cancerous or precancerous growths.

One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.

The clear fluid that travels through the lymphatic system and carries cells that help fight infections and other diseases. Also called lymphatic fluid.

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.

The flow of lymph from an area of tissue into a particular lymph node.

A thin tube that carries lymph (lymphatic fluid) and white blood cells through the lymphatic system. Also called lymphatic vessel.

A surgical procedure in which the lymph nodes are removed and examined to see whether they contain cancer. For a regional lymphadenectomy, some of the lymph nodes in the tumor area are removed; for a radical lymphadenectomy, most or all of the lymph nodes in the tumor area are removed. Also called lymph node dissection.

A type of cancer that begins in the cells that line lymph vessels.

The tissues and organs that produce, store, and carry white blood cells that fight infections and other diseases. This system includes the bone marrow, spleen, thymus, lymph nodes, and lymphatic vessels (a network of thin tubes that carry lymph and white blood cells). Lymphatic vessels branch, like blood vessels, into all the tissues of the body.

Cancerous. Malignant tumors can invade and destroy nearby tissue and spread to other parts of the body.

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.

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.

A type of soft tissue sarcoma that develops in cells that form a protective sheath (covering) around peripheral nerves, which are nerves that are outside of the central nervous system (brain and spinal cord). Also called MPNST.

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.

Refers to cells that develop into connective tissue, blood vessels, and lymphatic tissue.

The spread of cancer from one part of the body to another. A tumor formed by cells that have spread is called a “metastatic tumor” or a “metastasis.” The metastatic tumor contains cells that are like those in the original (primary) tumor. The plural form of metastasis is metastases (meh-TAS-tuh-SEEZ).

Having to do with metastasis, which is the spread of cancer from one part of the body to another.

In medicine, to regularly watch and check a person or condition to see if there is any change. Also refers to a device that records and/or displays patient data, such as for an electrocardiogram (EKG).

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.

The National Cancer Institute, part of the National Institutes of Health of the United States Department of Health and Human Services, is the Federal Government's principal agency for cancer research. The National Cancer Institute conducts, coordinates, and funds cancer research, training, health information dissemination, and other programs with respect to the cause, diagnosis, prevention, and treatment of cancer. Access the National Cancer Institute Web site at http://www.cancer.gov. Also called NCI.

NCI, part of the National Institutes of Health of the United States Department of Health and Human Services, is the Federal Government's principal agency for cancer research. It conducts, coordinates, and funds cancer research, training, health information dissemination, and other programs with respect to the cause, diagnosis, prevention, and treatment of cancer. Access the NCI Web site at http://www.cancer.gov. Also called National Cancer Institute.

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.

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.

Cancer that has not spread from the primary (original) site to other sites in the body.

A drug that decreases fever, swelling, pain, and redness. Also called NSAID.

A doctor who specializes in treating cancer. Some oncologists specialize in a particular type of cancer treatment. For example, a radiation oncologist specializes in treating cancer with radiation.

A part of the body that performs a specific function. For example, the heart is an organ.

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.

A specific result or effect that can be measured. Examples of outcomes include decreased pain, reduced tumor size, and improvement of disease.

A doctor who identifies diseases by studying cells and tissues under a microscope.

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.

Having to do with children.

A doctor who specializes in treating blood disorders in children.

A registered nurse with an advanced degree in nursing who specializes in the care of children.

A surgeon who specializes in the treatment of children. A surgeon removes or repairs a part of the body by operating on the patient.

An exam of the body to check for general signs of disease.

Occurring in various distinct forms. In terms of cells, having variation in the size and shape of cells or their nuclei.

The original tumor.

The likely outcome or course of a disease; the chance of recovery or recurrence.

Cancer that is growing, spreading, or getting worse.

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.

A specialist who can talk with patients and their families about emotional and personal matters, and can help them make decisions.

Energy released in the form of particle or electromagnetic waves. Common sources of radiation include radon gas, cosmic rays from outer space, medical x-rays, and energy given off by a radioisotope (unstable form of a chemical element that releases radiation as it breaks down and becomes more stable).

A doctor who specializes in using radiation to treat cancer.

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.

Giving off radiation.

A small, radioactive pellet that is placed in or near a tumor. Cancer cells are killed by the energy given off as the radioactive material breaks down and becomes more stable.

A molecule inside or on the surface of a cell that binds to a specific substance and causes a specific physiologic effect in the cell.

To come back or to return.

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.

Treatment with anticancer drugs directed to a specific area of the body.

A healthcare professional who helps people recover from an illness or injury and return to daily life. Examples of rehabilitation specialists are physical therapists and occupational therapists.

In medicine, an improvement related to treatment.

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).

Cancer that forms in the soft tissues in a type of muscle called striated muscle. Rhabdomyosarcoma can occur anywhere in the body.

Something that may increase the chance of developing a disease. Some examples of risk factors for cancer include age, a family history of certain cancers, use of tobacco products, certain eating habits, obesity, lack of exercise, exposure to radiation or other cancer-causing agents, and certain genetic changes.

A cancer of the bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue.

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.

Refers to a new primary cancer in a person with a history of cancer.

The first lymph node to which cancer is likely to spread from the primary tumor. When cancer spreads, the cancer cells may appear first in the sentinel node before spreading to other lymph nodes.

Removal and examination of the sentinel node(s) (the first lymph node(s) to which cancer cells are likely to spread from a primary tumor). To identify the sentinel lymph node(s), the surgeon injects a radioactive substance, blue dye, or both near the tumor. The surgeon then uses a scanner to find the sentinel lymph node(s) containing the radioactive substance or looks for the lymph node(s) stained with dye. The surgeon then removes the sentinel node(s) to check for the presence of cancer cells.

A problem that occurs when treatment affects healthy tissues or organs. Some common side effects of cancer treatment are fatigue, pain, nausea, vomiting, decreased blood cell counts, hair loss, and mouth sores.

A professional trained to talk with people and their families about emotional or physical needs, and to find them support services.

Refers to muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.

A cancer that begins in the muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.

The bones, muscles, tendons, and other tissues that reach from the base of the skull to the tailbone. The spinal column encloses the spinal cord and the fluid surrounding the spinal cord. Also called backbone, spine, and vertebral column.

A type of connective tissue cancer in which the cells are spindle-shaped when examined under a microscope.

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.

Performing exams and tests to learn the extent of the cancer within the body, especially whether the disease has spread from the original site to other parts of the body. It is important to know the stage of the disease in order to plan the best treatment.

In medicine, treatment that experts agree is appropriate, accepted, and widely used. Health care providers are obligated to provide patients with standard therapy. Also called best practice and standard of care.

A nonsteroidal anti-inflammatory drug (NSAID) that is being studied as a treatment for cancer.

Affecting cells on the surface. Not invasive.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

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.

A malignant tumor that develops in the synovial membrane of the joints.

Treatment with anticancer drugs that travel through the blood to cells all over the body.

A type of treatment that uses drugs or other substances, such as monoclonal antibodies, to identify and attack specific cancer cells without harming normal cells. Targeted therapy may be less harmful to normal cells than other types of cancer treatments

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.

Treatment.

An operation to open the chest.

A group or layer of cells that work together to perform a specific function.

A surgical procedure in which tissue or an organ is transferred from one area of a person’s body to another area, or from one person (the donor) to another person (the recipient).

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 cancerous), or malignant (cancerous). Also called neoplasm.

A drug that interferes with cell communication and growth and may prevent tumor growth. Some tyrosine kinase inhibitors are used to treat cancer.

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.

A blood vessel that carries blood to the heart from tissues and organs in the body.

Necessary to maintain life. Breathing is a vital function.

Closely monitoring a patient's condition but withholding treatment until symptoms appear or change. Also called active surveillance, expectant management, and observation.

Refers to a blood cell that does not contain hemoglobin. White blood cells include lymphocytes, neutrophils, eosinophils, macrophages, and mast cells. These cells are made by bone marrow and help the body fight infections and other diseases. Also called leukocyte and WBC.

Surgery to cut out the cancer and some healthy tissue around it.

A type of high-energy radiation. 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.