ご利用について
This PDQ cancer information summary has current information about the treatment of childhood soft tissue sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Date Last Modified") is the date of the most recent change. The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
CONTENTS
- What is childhood soft tissue sarcoma?
-
Childhood soft tissue sarcoma is cancer that forms in muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.
Soft tissues of the body connect, support, and surround other body parts and organs. The soft tissues include:
Soft tissue sarcoma may occur anywhere in the body. In children, the tumors form most often in the arms, legs, chest, or abdomen.
Soft tissue sarcoma forms in the soft tissues of the body, including the muscles, tendons, ligaments, cartilage, fat, blood vessels, lymph vessels, nerves, and tissues around joints. Soft tissue sarcoma occurs in children and adults. Soft tissue sarcoma in children may respond differently to treatment and may have a better prognosis than soft tissue sarcoma in adults. Learn more about soft tissue sarcoma in adults at Soft Tissue Sarcoma Treatment.
- Types of soft tissue sarcoma
-
There are many types of soft tissue sarcoma. They are grouped based on the type of soft tissue cell where they first formed.
This summary is about the following types of soft tissue sarcoma:
Blood vessel tumors
Blood vessel tumors include the following types:
Bone tumors
Extraskeletal osteosarcoma. This type of bone and cartilage cancer is very rare in children and adolescents. It is likely to come back after treatment and may spread to the lungs. Osteosarcoma occurs more often in bone.
Cartilage tumors
Extraskeletal mesenchymal chondrosarcoma. This type of cartilage cancer often affects young adults and occurs in the head and neck. It is usually high grade (likely to grow quickly) and may spread to other parts of the body. It may also come back many years after treatment.
Fat tissue tumors
Liposarcoma. This is a cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In children and adolescents, liposarcoma is often low grade (likely to grow and spread slowly). Liposarcoma may spread to the lungs and rarely to the lymph nodes. There are several different types of liposarcoma, including:
Fibrous connective tissue tumors
Fibrous connective tissue tumors have several subtypes, including:
Pericytic (perivascular) tumors
Pericytic tumors form in cells that wrap around blood vessels. There are different types of pericytic tumors, including:
Peripheral nerve sheath tumor
The nerve sheath is made up of protective layers of myelin that cover nerve cells that are not part of the brain or spinal cord. Learn about Malignant Peripheral Nerve Sheath Tumor.
Skeletal muscle tumors
Skeletal muscle is attached to bones and helps the body move.
Smooth muscle tumors
Smooth muscle lines the inside of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus. Learn about Leiomyosarcoma.
So-called fibrohistiocytic tumors
Tumors of unknown cell origin
Tumors of unknown cell origin (the type of cell the tumor first formed in is not known) have many subtypes, including:
- Causes and risk factors for childhood soft tissue sarcoma
-
Childhood soft tissue sarcoma is caused by certain changes to the way soft tissue cells function, especially how they grow and divide into new cells. Often, the exact cause of these changes is unknown. Learn more about how cancer develops at What Is Cancer?
A risk factor is anything that increases the chance of getting a disease. Not every child with one or more of these risk factors will develop a soft tissue sarcoma. And it will develop in some children who don't have a known risk factor.
The risk of soft tissue sarcoma may be increased if your child has one of the following inherited disorders:
Another risk factor is past treatment with radiation therapy.
Talk with your child's doctor if you think your child may be at risk.
- Symptoms of childhood soft tissue sarcoma
-
Soft tissue sarcoma may appear as a painless lump under the skin, often on an arm, a leg, the chest, or the abdomen. There may be no other symptoms at first. As the sarcoma gets bigger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause symptoms, such as pain or weakness. Fever, weight loss, night sweats, and low or high blood sugar levels are rare symptoms.
Other conditions may cause the same symptoms. Check with your child's doctor if your child has any of these problems.
- Tests to diagnose childhood soft tissue sarcoma
-
If your child has symptoms that suggest a soft tissue sarcoma, the doctor will need to find out if these are due to cancer or another problem. The doctor will ask when the symptoms started and how often your child has been having them. They will also ask about your child's personal and family medical history and do a physical exam. Depending on these results, they may recommend tests to find out if your child has a soft tissue sarcoma, and if so, its extent (stage).
The following tests may be used to diagnose soft tissue sarcomas. The results of these tests will also help you and your child's doctor plan treatment.
X-ray
X-ray is a type of radiation that can go through the body and make pictures of areas inside the body.
Magnetic resonance imaging (MRI)
MRI uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas in the body, such as the chest, abdomen, arms, or legs. This procedure is also called nuclear magnetic resonance imaging (NMRI).
Magnetic resonance imaging (MRI) scan. The child lies on a table that slides into the MRI machine, which takes a series of detailed pictures of areas inside the body. The positioning of the child on the table depends on the part of the body being imaged. CT scan
CT scan (CAT scan) uses a computer linked to an x-ray machine to make a series of detailed pictures of areas inside the body, such as the chest or abdomen. The pictures are taken from different angles and are used to create 3-D views of tissues and organs. 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. Learn more about Computed Tomography (CT) Scans and Cancer.
Computed tomography (CT) scan. The child lies on a table that slides through the CT scanner, which takes a series of detailed x-ray pictures of areas inside the body. Ultrasound
Ultrasound uses high-energy sound waves (ultrasound) that bounce off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram.
Biopsy
Biopsy is the removal of a sample of cells or tissue from the tumor so that a pathologist can view it under a microscope to check for cancer. The type of biopsy depends, in part, on the size of the mass and whether it is close to the surface of the skin or deeper in the tissue. The following types of biopsies may be used to check for soft tissue sarcoma:
If possible, it is important to have the same surgeon perform the biopsy and the surgery to remove the tumor. The placement of needles or incisions for the biopsy can affect whether the tumor can be completely removed during a future surgery.
To plan the best treatment, the sample of tissue removed during the biopsy must be large enough to find out the type of soft tissue sarcoma and do other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. 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.
One or more of the following laboratory tests may be done to study the tissue samples:
The following tests may be used to find out if cancer has spread:
Sentinel lymph node biopsy
Sentinel lymph node biopsy removes the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node in a group of lymph nodes to receive lymphatic drainage from the primary tumor. It is therefore the first lymph node the cancer is likely to spread to from the primary tumor. To identify the sentinel lymph node, a radioactive substance, blue dye, or both 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 found, more lymph nodes will be removed through a separate incision (cut). This is called a lymph node dissection. Sometimes, a sentinel lymph node is found in more than one group of nodes. This procedure is used for epithelioid sarcoma.
PET-CT scan
PET-CT scan combines the pictures from a positron emission tomography (PET) scan and a computed tomography (CT) scan. The PET and CT scans are done at the same time on the same machine. The combined scans make more detailed pictures than either test would make by itself.
- Getting a second opinion
-
You may want to get a second opinion to confirm your child's cancer diagnosis and treatment plan. If you seek a second opinion, you will need to get medical test results and reports from the first doctor to share with the second doctor. The second doctor will review the genetic test results, pathology report, slides, and scans. This doctor may agree with the first doctor, suggest changes or another approach, or provide more information about your child's tumor.
To learn more about choosing a doctor and getting a second opinion, visit Finding Cancer Care. You can contact NCI's Cancer Information Service via chat, email, or phone (both in English and Spanish) for help finding a doctor or hospital that can provide a second opinion. For questions you might want to ask at your appointments, visit Questions to Ask Your Doctor about Cancer.
- Types of treatment for childhood soft tissue sarcoma
-
Who treats children with soft tissue sarcoma?
A pediatric oncologist, a doctor who specializes in treating children with cancer, oversees treatment for soft tissue sarcoma. 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. Other specialists may include:
There are different types of treatment for children and adolescents with soft tissue sarcoma. You and your child's cancer care team will work together to decide treatment. Often, the treatments depend on the type of soft tissue sarcoma. Many other factors will be considered, such as your child's overall health and whether the cancer is newly diagnosed or has come back.
Your child's treatment plan will include information about the cancer, the goals of treatment, treatment options, and the possible side effects. It will be helpful to talk with your child's cancer care team before treatment begins about what to expect. For help every step of the way, visit our booklet, Children with Cancer: A Guide for Parents.
Types of treatment your child might have include:
Surgery
Surgery to completely remove the soft tissue sarcoma is done when 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. This is called neoadjuvant (preoperative) therapy.
The following types of surgery may be used:
A second-look surgery may be needed to:
After the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy 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.
Learn more about Surgery to Treat Cancer.
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 different types of radiation therapy:
The way radiation therapy is given depends on the type and stage of the cancer being treated, if any cancer cells remain after surgery, and the expected side effects of treatment. External and internal radiation therapy are used to treat childhood soft tissue sarcoma.
Learn more about Radiation Therapy to Treat Cancer.
Chemotherapy
Chemotherapy (also called chemo) uses drugs to stop the growth of cancer cells. Chemotherapy either kills the cancer cells or stops them from dividing. Chemotherapy may be given alone or with other types of treatment, such as radiation therapy.
For children with soft tissue sarcoma, chemotherapy is taken by mouth or injected into a vein. When given this way, the drugs enter the bloodstream to reach cancer cells throughout the body.
The way the chemotherapy is given depends on the type of soft tissue sarcoma being treated. Some types of soft tissue sarcoma may respond to treatment with chemotherapy.
Chemotherapy that may be used alone or in combination includes:
Other chemotherapy drugs not listed here may also be used.
Learn more about how chemotherapy works, how it is given, common side effects, and more at Chemotherapy to Treat Cancer.
Observation
Observation is closely monitoring a person's condition without giving any treatment until signs or symptoms appear or change. Observation may be done when:
Observation may be used for epithelioid hemangioendothelioma, infantile fibrosarcoma, or PEComa.
Targeted therapy
Targeted therapy uses drugs or other substances to block the action of specific enzymes, proteins, and other molecules involved in the growth and spread of cancer cells. Targeted therapies used to treat soft tissue sarcoma include:
Learn more about Targeted Therapy to Treat Cancer.
Immunotherapy
Immunotherapy is a treatment that uses the person's immune system to fight cancer. Pembrolizumab may be used to treat undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma) and progressive and recurrent soft tissue sarcoma.
Learn more about Immunotherapy to Treat Cancer.
Clinical trials
For some children, joining a clinical trial may be an option. There are different types of clinical trials for childhood cancer. For example, a treatment trial tests new treatments or new ways of using current treatments. Supportive care and palliative care trials look at ways to improve quality of life, especially for those who have side effects from cancer and its treatment.
You can use the clinical trial search to find NCI-supported cancer clinical trials accepting participants. The search allows you to filter trials based on the type of cancer, your child's age, and where the trials are being done. Clinical trials supported by other organizations can be found on the ClinicalTrials.gov website.
Learn more about clinical trials, including how to find and join one, at Clinical Trials Information for Patients and Caregivers.
- Treatment of childhood soft tissue sarcoma
-
To learn about the treatments listed below, visit Types of treatment for childhood soft tissue sarcoma.
Blood vessel tumors
Epithelioid hemangioendothelioma
Treatment of newly diagnosed epithelioid hemangioendothelioma may include:
Angiosarcoma
Treatment of newly diagnosed angiosarcoma may include:
Bone tumors
Extraskeletal osteosarcoma
Treatment of newly diagnosed extraskeletal osteosarcoma is surgery to completely remove the tumor, followed by chemotherapy.
Learn more at Osteosarcoma Treatment.
Cartilage tumors
Extraskeletal mesenchymal chondrosarcoma
Treatment of newly diagnosed extraskeletal mesenchymal chondrosarcoma may include:
Fat tissue tumors
Liposarcoma
Treatment of newly diagnosed liposarcoma may include:
Fibrous connective tissue tumors
Dermatofibrosarcoma protuberans
Treatment of newly diagnosed dermatofibrosarcoma protuberans may include:
Fibrosarcoma
Infantile fibrosarcoma
Treatment of newly diagnosed infantile fibrosarcoma may include:
Adult-type fibrosarcoma
Treatment of adult-type newly diagnosed fibrosarcoma is surgery to completely remove the tumor, when possible.
Myxofibrosarcoma
Treatment of newly diagnosed myxofibrosarcoma is surgery to completely remove the tumor.
Low-grade fibromyxoid sarcoma
Treatment of newly diagnosed low-grade fibromyxoid sarcoma is surgery to completely remove the tumor.
Pericytic (perivascular) tumors
Infantile hemangiopericytoma
Treatment of newly diagnosed infantile hemangiopericytoma is usually chemotherapy.
Skeletal muscle tumors
Rhabdomyosarcoma
Learn more at Childhood Rhabdomyosarcoma Treatment.
Ectomesenchymoma
Treatment of newly diagnosed ectomesenchymoma may include:
So-called fibrohistiocytic tumors
Plexiform fibrohistiocytic tumor
Treatment of newly diagnosed plexiform fibrohistiocytic tumor is surgery to completely remove the tumor.
Tumors of unknown cell origin (the place where the tumor first formed is not known)
Myxoma (Carney complex)
Other summaries with information related to Carney complex include:
Epithelioid sarcoma
Treatment of newly diagnosed epithelioid sarcoma may include:
Extraskeletal myxoid chondrosarcoma
Treatment of newly diagnosed extraskeletal myxoid chondrosarcoma may include:
Extraskeletal Ewing sarcoma
Learn more at Ewing Sarcoma Treatment.
Perivascular epithelioid cell tumors (PEComas)
Treatment of newly diagnosed perivascular epithelioid cell tumors may include:
Undifferentiated or unclassified sarcoma
Undifferentiated pleomorphic sarcoma (malignant fibrous histiocytoma)
Treatment of newly diagnosed undifferentiated pleomorphic sarcoma may include surgery. Treatment of recurrent or refractory undifferentiated pleomorphic sarcoma may include immunotherapy (pembrolizumab).
Learn more about the treatment of malignant fibrous histiocytoma of bone at Osteosarcoma Treatment.
Metastatic childhood soft tissue sarcoma
Treatment of childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis is chemotherapy and radiation therapy. Surgery may be done to remove tumors that have spread to the lung.
To learn more about the treatment of specific tumor types, visit Treatment of Childhood Soft Tissue Sarcoma.
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
- Treatment of progressive or recurrent childhood soft tissue sarcoma
-
To learn about the treatments listed below, visit Types of treatment for childhood soft tissue sarcoma.
Treatment of progressive or recurrent childhood soft tissue sarcoma may include:
Use our clinical trial search to find NCI-supported cancer clinical trials that are accepting patients. You can search for trials based on the type of cancer, the age of the patient, and where the trials are being done. General information about clinical trials is also available.
- Side effects and late effects of treatment
-
Cancer treatments can cause side effects. Which side effects your child might have depends on the type of treatment they receive, the dose, and how their body reacts. Talk with your child's treatment team about which side effects to look for and ways to manage them.
To learn more about side effects that begin during treatment for cancer, visit Side Effects.
Problems from cancer treatment that begin 6 months or later after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:
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. Learn more about Late Effects of Treatment for Childhood Cancer.
- Prognostic factors for childhood soft tissue sarcoma
-
If your child has been diagnosed with a soft tissue sarcoma, you may have questions about how serious the cancer is and your child's chances of survival. The likely outcome or course of a disease is called prognosis. The prognosis depends on:
No two people are alike, and responses to treatment can vary greatly. Your child's cancer care team is in the best position to talk with you about your child's prognosis.
- Follow-up care
-
As your child goes through treatment, they will have follow-up tests or check-ups. Some of the tests that were done to diagnose the cancer may be repeated 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.
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 child's condition has changed or if the cancer has recurred (come back).
Learn more about the follow-up tests in Tests to diagnose childhood soft tissue sarcoma.
- Coping with your child's cancer
When your child has cancer, every member of the family needs support. Taking care of yourself during this difficult time is important. Reach out to your child's treatment team and to people in your family and community for support. To learn more, visit Support for Families: Childhood Cancer and the booklet Children with Cancer: A Guide for Parents.
- Related resources
-
For more childhood cancer information and other general cancer resources, visit:
- About This PDQ Summary
-
About PDQ
Physician Data Query (PDQ) is the National Cancer Institute's (NCI's) comprehensive cancer information database. 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 come in two versions. The health professional versions have detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions have cancer information that is accurate and up to date and most versions are also available in Spanish.
PDQ is a service of the NCI. The NCI is part of the National Institutes of Health (NIH). NIH is the federal government’s center of biomedical research. The PDQ summaries are based on an independent review of the medical literature. They are not policy statements of the NCI or the NIH.
Purpose of This Summary
This PDQ cancer information summary has current information about the treatment of childhood soft tissue sarcoma. It is meant to inform and help patients, families, and caregivers. It does not give formal guidelines or recommendations for making decisions about health care.
Reviewers and Updates
Editorial Boards write the PDQ cancer information summaries and keep them up to date. These Boards are made up of experts in cancer treatment and other specialties related to cancer. The summaries are reviewed regularly and changes are made when there is new information. The date on each summary ("Updated") is the date of the most recent change.
The information in this patient summary was taken from the health professional version, which is reviewed regularly and updated as needed, by the PDQ Pediatric Treatment Editorial Board.
Clinical Trial Information
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." Patients may want to think about taking part in a clinical trial. Some clinical trials are open only to patients who have not started treatment.
Clinical trials can be found online at NCI's website. For more information, call the Cancer Information Service (CIS), NCI's contact center, at 1-800-4-CANCER (1-800-422-6237).
Permission to Use This Summary
PDQ is a registered trademark. The content of PDQ documents can be used freely as text. It cannot be identified as an NCI PDQ cancer information summary unless the whole summary is shown and it is updated regularly. However, a user would be allowed to write a sentence such as “NCI’s PDQ cancer information summary about breast cancer prevention states the risks in the following way: [include excerpt from the summary].”
The best way to cite this PDQ summary is:
PDQ® Pediatric Treatment Editorial Board. PDQ Childhood Soft Tissue Sarcoma. Bethesda, MD: National Cancer Institute. Updated <MM/DD/YYYY>. Available at: https://www.cancer.gov/types/soft-tissue-sarcoma/patient/child-soft-tissue-treatment-pdq. Accessed <MM/DD/YYYY>. [PMID: 26389342]
Images in this summary are used with permission of the author(s), artist, and/or publisher for use in the PDQ summaries only. If you want to use an image from a PDQ summary and you are not using the whole summary, you must get permission from the owner. It cannot be given by the National Cancer Institute. Information about using the images in this summary, along with many other images related to cancer can be found in Visuals Online. Visuals Online is a collection of more than 3,000 scientific images.
Disclaimer
The information in these summaries should not be used to make decisions about insurance reimbursement. More information on insurance coverage is available on Cancer.gov on the Managing Cancer Care page.
Contact Us
More information about contacting us or receiving help with the Cancer.gov website can be found on our Contact Us for Help page. Questions can also be submitted to Cancer.gov through the website’s E-mail Us.