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Sarcoma - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2013

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Sarcoma. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

Cancer begins when normal cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).

About sarcoma

Sarcoma can start in any part of the body, such as the bone or soft tissue. About 60% of soft tissue sarcomas begin in an arm or leg, 30% start in the trunk (torso) or abdomen, and 10% occur in the head or neck. Both children and adults can develop a sarcoma, but it is rare in adults, accounting for about 1% of all adult cancers. However, sarcoma in general represents about 15% of all cancers in children.

This section covers sarcoma of the soft tissues. Learn more about sarcoma that starts in a bone.

About soft-tissue sarcoma

Soft-tissue sarcomas (STS) are a group of cancers that begin in the tissues that support and connect the body, such as fat cells, muscle, nerves, tendons, the lining of joints, blood vessels, or lymph vessels. As a result, STS can occur almost anywhere in the body. When an STS is small, it can go unnoticed, since it does not usually cause problems early on. However, as an STS grows, it can interfere with the body's normal activities.

Because there are several different types of STS, it is more of a family of related diseases rather than a single, specific disease. The specific types of sarcoma are often named according to the normal tissue cells they most closely resemble (see below). This is different from most other types of cancer, which are usually named for the part of the body where the cancer began. Some sarcomas do not look like any type of normal tissue and are thought to come from stem cells, which are special cells that can mature into specific tissues or organs.

Name of Sarcoma

Related Normal Tissue Type

Angiosarcoma

Blood or lymph vessels

Ewing Family of sarcomas (peripheral primitive neuroectodermal tumor, PNET)

 

 

No obvious related normal tissue; may be a tumor of stem cells

Fibrosarcoma

Fibrous tissue (tendons and ligaments)

Gastrointestinal stromal tumor (GIST)

Specialized neuro-muscular cells of the digestive tract

Kaposi sarcoma

Blood vessels

Leiomyosarcoma

Smooth muscle

Liposarcoma

Fat tissue

Myxofibrosarcoma (myxoid malignant fibrous histiocytoma, MFH)

 

Connective tissue

Malignant peripheral nerve sheath tumor (MPNST, also known as neurofibrosarcoma)

Cells that wrap around nerve endings similar to how insulation wraps around a wire

Pleomorphic sarcoma, not otherwise specified (NOS; often referred to in the past as malignant fibrous histiocytoma, MFH)

 

No obvious related normal tissue; may be a tumor of stem cells

Rhabdomyosarcoma

Skeletal muscle

Synovial sarcoma

No obvious related normal tissue; may be a tumor of stem cells

The list above is not complete, but several of the most common types are listed. Experts have found many types and subtypes of sarcomas. Pathologists (doctors who specialize in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease) are now trying to find new ways to quickly determine a tumor's subtype, as this helps determine treatment. Looking at a tumor's abnormal genetics may help determine its characteristics and predict which treatments will be most effective. For at least two types of sarcoma, GIST and dermatofibrosarcoma protuberans (DFSP), major advances have been made in a type of treatment called targeted therapy (see Treatment). 

Grade is another characteristic pathologists use to describe sarcomas. The grade helps predict how aggressive the sarcoma is likely to be, meaning how likely it will grow and spread to another part of the body. A low-grade tumor generally grows more slowly and often stays in the area where it started. A high-grade tumor is more likely to spread to other places, called metastasis. Learn more about grade in the Staging section.

Find out more about basic cancer terms used in this section.

Looking for More of an Overview?

If you would like additional introductory information, explore these related items on Cancer.Net:

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Sarcoma - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2013

ON THIS PAGE: You will find information about how many people learn they have this type of cancer each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

This year, approximately 12,020 people (6,550 males and 5,470 females) will be diagnosed with soft-tissue sarcoma in the United States. An estimated 4,740 adults and children (2,550 males and 2,190 females) are expected to die of the disease this year.

The five-year relative survival rate is the percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases. The five-year survival rate of people with localized sarcomas is 83%. For sarcomas that have spread to regional lymph nodes, the five-year survival rate is 54%. For sarcomas that have spread to distant parts of the body, the five-year survival is 16%.  However, when the sarcoma is located in an arm or leg, the five-year survival rates are slightly higher for each stage.

How long a person lives with sarcoma depends on many factors, including the size of the tumor, where it is located, the type, the mitotic rate (how fast the tumor cells are growing and dividing), and whether it is superficial or deep (see Staging). If the sarcoma is diagnosed at an early stage and hasn’t spread from where it started, treatment is very effective and many people can be cured. On the other hand, if the sarcoma has spread to other parts of the body, treatment can usually control the tumor, but it is not often curable.

Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of people with this type of cancer, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with sarcoma. Because the survival statistics are measured in five-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer. Learn more about understanding statistics.

Statistics adapted from the American Cancer Society's publication, Cancer Facts & Figures 2014.

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Sarcoma - Medical Illustrations

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2013

ON THIS PAGE: You will find a basic drawing bout the main body parts affected by this disease. To see other pages, use the menu on the side of your screen.

Soft Tissue Anatomy

Larger image

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Sarcoma - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2013

ON THIS PAGE: You will find out more about the factors that increase the chance of developing this type of cancer. To see other pages, use the menu on the side of your screen.

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

Most sarcomas do not have known causes. The following factors can raise a person’s risk of developing sarcoma:

Previous radiation therapy. People who have been treated with radiation therapy for a previous cancer have a slightly increased risk of later developing sarcoma.

Genetics. People with certain inherited diseases, such as von Recklinghausen’s disease (neurofibromatosis), Gardner syndrome, Werner syndrome, tuberous sclerosis, nevoid basal cell carcinoma syndrome, Li-Fraumeni syndrome, or retinoblastoma have a higher risk of sarcoma.

Chemicals. Workplace exposure to vinyl chloride monomer (used in making some types of plastics) or to dioxin may increase the risk of sarcoma. However, most sarcoma is not known to be associated with specific environmental hazards.

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Sarcoma - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2013

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

People with sarcoma may experience the following symptoms or signs. Sometimes, people with sarcoma do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign on this list, please talk with your doctor.         

STS rarely causes symptoms in the early stages. The first sign of a sarcoma in an arm, leg, or trunk may be a painless lump or swelling. Most lumps are not sarcoma. The most common soft-tissue lumps are lipomas, which are made of fat cells and are not cancer. These lumps have often been there for many years and do not change in size. It’s important to talk with your doctor about any lumps that get larger or are painful. People with sarcoma that starts in the abdomen may not have any symptoms or may have pain or a sense of fullness.

Because STS can develop in flexible, elastic tissues or deep spaces in the body, the tumor can often easily push normal tissue out of its way as it grows. Therefore, a sarcoma may grow quite large before it causes symptoms. Eventually, it may cause pain as the growing tumor begins to press against nerves and muscles.

Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptom(s) and how often.

If sarcoma is diagnosed, relieving symptoms and side effects remains an important part of cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.

The next section helps explain what tests and scans may be needed to learn more about the cause of the symptoms. Use the menu on the side of your screen to select Diagnosis, or you can select another section, to continue reading this guide.  

Sarcoma - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2013

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

Doctors use many tests to diagnose cancer and find out if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of cancer, including sarcoma, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has metastasized. Your doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition
  • Type of cancer suspected
  • Severity of symptoms
  • Previous test results

There are no standard screening tests for sarcoma. A doctor should examine any unusual or new lumps or bumps that are growing to make sure it is not cancer. Sarcoma is rare. This makes it important to talk with a doctor who has experience with this type of cancer if sarcoma is suspected.

A diagnosis of sarcoma is made by a combination of clinical examination by a doctor and imaging tests. It is confirmed by the results of a biopsy. In addition to a physical examination, the following tests may be used to diagnose sarcoma:

Imaging tests

A benign and a cancerous tumor may look different on imaging tests, such as an x-ray. In general, a benign tumor has round, smooth, well-defined borders. A cancerous tumor has irregular, poorly defined edges.

X-ray. An x-ray is a way to create a picture of the structures inside of the body, using a small amount of radiation. Typically, if an x-ray suggests cancer, the doctor will order other imaging tests. X-ray is particularly useful for bone sarcomas.

Ultrasound. An ultrasound uses sound waves to create a picture of the internal organs.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a patient’s vein to provide better detail.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture. MRI scans are useful to check for a tumor in soft tissues.

Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive substance is injected into a patient’s body. This substance is absorbed mainly by organs and tissues that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the tumor inside of the body.

Integrated PET-CT scan. This scanning method collects images from both a CT and PET scan at the same time, and then combines the images. This technique can be used to look at both the structure and how much energy is used by the tumor and normal tissues. This information can be helpful in treatment planning and evaluating how well treatment is working.

Imaging tests may suggest the diagnosis of sarcoma, but a biopsy will always be performed to confirm the diagnosis and find out the subtype. It is very important for a patient to see a sarcoma specialist before any surgery or biopsy is done.

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed from the biopsy is analyzed by a pathologist.

There are different types of biopsies. For a needle biopsy (usually a core needle biopsy, less often a thin needle biopsy), a doctor removes a small sample of tissue from the tumor with a needle-like instrument. This may be performed with the help of ultrasound, CT scan, or MRI to precisely find the tumor. In an incisional biopsy, the surgeon cuts into the tumor and removes a sample of tissue. In an excisional biopsy, the surgeon removes the entire tumor. Because these tumors are uncommon, it is important that the sample of tissue removed is reviewed by an expert pathologist to appropriately diagnose a sarcoma.

Molecular testing of the tumor. Your doctor may recommend running laboratory tests on a tumor sample to identify specific genes, proteins, and other factors unique to the tumor. Results of these tests will help decide whether your treatment options include a type of treatment called targeted therapy (see Treatment).

Learn more about what to expect when having common tests, procedures, and scans.

After these diagnostic tests are done, your doctor will review all of the results with you. If the diagnosis is cancer, these results also help the doctor describe the cancer; this is called staging. Learn more about the first steps to take after a diagnosis of cancer

The next section helps explain the different stages for this type of cancer. Use the menu on the side of your screen to select Stages, or you can select another section, to continue reading this guide.  

Sarcoma - Stages

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2013

ON THIS PAGE: You will learn about how doctors describe a cancer’s growth or spread. This is called the stage. To see other pages, use the menu on the side of your screen.

Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). There are different stage descriptions for different types of cancer.

There are a number of systems used to find out the stage of sarcoma. These systems are somewhat different from each other, but all use an overall stage to help predict a person’s prognosis.

One tool that doctors use to describe the stage of common cancers is known as the TNM system. This system judges three factors: the tumor itself, the lymph nodes around the tumor, and if the tumor has spread to the rest of the body. The results are combined to determine the stage of cancer for each person. There are four stages: stages I through IV (one through four). The stage provides a common way of describing the cancer, so doctors can work together to plan the best treatments.

TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer:

  • How large is the primary tumor and where is it located? (Tumor, T)
  • Has the tumor spread to the lymph nodes? (Node, N)
    Important note: Most sarcomas (except for rare exceptions, such as epithelioid sarcoma, some subtypes of rhabdomyosarcoma, and clear cell sarcoma) do not spread to lymph nodes, so surgeons do not often remove and test lymph nodes for most types of sarcoma.
  • Has the cancer metastasized to other parts of the body? (Metastasis, M)

When staging sarcoma, another factor, grade (G), is added to the TNM system. This is different than staging for many other types of cancer.

Tumor. Using the TNM system, the "T" plus a number (0 to 2) and letter (a or b) is used to describe the size and location of the tumor. The tumor (T) is further described by its depth. The tumor depth is described as either superficial (the tumor is above the body's superficial tissue) or deep (the tumor is either beneath the body's superficial tissue or invades the deep tissue). Specific tumor stage information is listed below.

TX: The primary tumor cannot be evaluated.

T0: There is no evidence of a primary tumor.

T1: The size of the tumor is 5 centimeters (cm) or smaller.

  • T1a: The tumor is superficial.
  • T1b: The tumor is deep.

T2: The size of the tumor is larger than 5 cm.

  • T2a: The tumor is superficial.
  • T2b: The tumor is deep.

Node. The "N" in the TNM staging system stands for lymph nodes, the tiny, bean-shaped organs located all over the body that normally help fight infections and cancer as part of the body's immune system. Lymph nodes near where the cancer started are called regional lymph nodes. Lymph nodes in other parts of the body are called distant lymph nodes. As noted above, lymph node involvement is unusual for soft tissue sarcoma.

NX: The regional lymph nodes cannot be evaluated.

N0: The cancer has not spread to the regional lymph nodes.

N1: The cancer has spread to the regional lymph nodes.

Metastasis. The "M" in the TNM system indicates whether the cancer has spread to other parts of the body.

M0: The cancer has not metastasized.

M1: There is metastasis to another part of the body.

Grade (G). The grade for sarcoma describes how aggressive a tumor is and how likely it is to grow and spread. A tumor's grade is described using the letter “G” and a number. There are four grades for sarcoma: GX (the grade cannot be evaluated), G1, G2, and G3. The grade is determined by calculating a score based on the following factors:

  • How different the cancer cells are from normal tissue cells
  • How many tumor cells are dividing
  • How much of the tumor has cells that are dying

The lower the combined score for these three factors, the lower the grade, meaning that the tumor is less aggressive and a patient’s prognosis is better. This is the recommended grading system, but there are others that may also be used. For instance, some doctors grade sarcomas as either “low grade” or “high grade,” particularly if the tumor has been treated before surgery.

Cancer stage grouping

Doctors assign the stage of the cancer by combining the T, N, and M classifications, along with G. For sarcoma, stages I and II are described in smaller groups according to the tumor's depth.

Stage IA: The tumor is 5 cm or less in size, and either superficial or deep. It has not spread to lymph nodes or to other parts of the body (T1a or T1b, N0, M0, GX or G1).

Stage IB: The tumor is more than 5 cm in size, and either superficial or deep. It has not spread to lymph nodes or to other parts of the body (T2a or T2b, N0, M0, GX or G1).

Stage IIA: The tumor is 5 cm or less in size, and either superficial or deep. It has not spread to lymph nodes or to other parts of the body. The grade is higher than the grade for stage IA (T1a or T1b, N0, M0, G2 or G3).

Stage IIB: The tumor is more than 5 cm in size, and either superficial or deep. It has not spread to lymph nodes or to other parts of the body. The grade is higher than the grade for stage IB (T2a or T2b, N0, M0, G2).

Stage III: This stage can be described by either of the following:

  • The tumor is more than 5 cm in size, and either superficial or deep. It has not spread to lymph nodes or to other parts of the body. The grade is higher than the grade for stage IIB (T2a or T2b, N0, M0, G3).
  • The tumor is any size, either superficial or deep, or any grade. It has spread to the regional lymph nodes but not to other parts of the body (any T, N1, M0, any G).

Stage IV: The tumor is any size, either superficial or deep, any grade, and may or may not have spread to the regional lymph nodes. It has spread to other parts of the body (any T, any N, M1, and any G).

This staging system applies to most types of soft tissue sarcoma except Kaposi sarcoma, GIST, and infantile fibrosarcoma. Also excluded from this staging system are sarcomas that begin in the central nervous system (including the brain or the lining around the brain and spinal cord), and sarcomas that begin in an organ or a hollow organ, such as the bowel or urinary tract.

Recurrent: Recurrent cancer is cancer that comes back after treatment. If there is a recurrence, the cancer may need to be staged again (called re-staging) using the system above.

Used with permission of the American Joint Committee on Cancer (AJCC), Chicago, Illinois. The original source for this material is the AJCC Cancer Staging Manual, Seventh Edition (2010) published by Springer-Verlag New York, www.cancerstaging.net.

Information about the cancer’s stage will help the doctor recommend a treatment plan.  The next section helps explain the treatment options for this type of cancer. Use the menu on the side of your screen to select Treatment Options, or you can select another section, to continue reading this guide.  

Sarcoma - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2013

ON THIS PAGE: You will learn about the different ways doctors use to treat people with this type of cancer. To see other pages, use the menu on the side of your screen.

This section outlines treatments that are the standard of care (the best proven treatments available) for this specific type of cancer. When making treatment plan decisions, patients are also encouraged to consider clinical trials as an option. A clinical trial is a research study to test a new treatment to evaluate whether it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, see the Clinical Trials and Current Research sections.

Treatment overview

In cancer care, different types of doctors often work together to create a patient’s overall treatment plan that combines different types of treatments. This is called a multidisciplinary team.

Descriptions of the most common treatment options for sarcoma are listed below. Treatment options and recommendations depend on several factors, including the type and stage of cancer, possible side effects, and the patient’s preferences and overall health. Learn more about making treatment decisions.

Surgery is the most common first treatment for sarcomas that are smaller and in specific locations. If the tumor cannot be removed by surgery, it may be possible to permanently control its growth with radiation therapy. For a tumor that can be surgically removed, radiation therapy and/or chemotherapy may be given before or after surgery to reduce the risk of recurrence (cancer coming back after treatment). Chemotherapy and radiation therapy may also be used to reduce the size of the sarcoma or relieve pain and other symptoms.

Surgery

Surgery is the removal of the tumor and surrounding tissue during an operation.

Before surgery, it’s important to have a biopsy to confirm the diagnosis (see Diagnosis). After a biopsy, surgery is typically the first and main treatment for an STS that is smaller and located in only one area. A surgical oncologist is a doctor who specializes in treating cancer using surgery. Sometimes, when an STS is on a limb (arm or leg) or spine, an orthopedic oncologist (a doctor who specializes in treating people with bone and soft tissue tumors) may perform surgery to remove the tumor.

The surgeon's goal is to remove the tumor and enough normal tissue around it to leave behind what is called a “clean margin” (meaning there are no tumor cells left in the area where the tumor was removed). Small sarcomas can usually be effectively eliminated by surgery alone. Those larger than 5 cm are often treated with a combination of surgery and radiation therapy. Radiation therapy or chemotherapy may be used before surgery (to shrink the tumor and make removal easier), or during and after surgery (to kill any remaining cancer cells). Learn more about cancer surgery.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time.

Radiation therapy may be done before surgery to shrink the tumor so it is more easily removed. Or, it may be done after surgery to remove any cancer cells left behind. Radiation treatment may make it possible to do less surgery, often preserving the arm or leg if that is where the sarcoma is located. Radiation therapy can also damage normal cells, but because it is focused around the tumor, side effects are usually limited to those areas.

In the short term, radiation can cause injury to the skin that looks like a sunburn and is usually treated with creams that keep the skin soft and helps relieve pain. In the long term, radiation can cause scarring that limits the function of an arm or a leg. In rare cases, radiation can cause a sarcoma or other cancer. Each person is encouraged to talk with his or her doctor about the possible risks and benefits of a specific treatment such as radiation therapy.

Most radiation therapy side effects go away soon after treatment ends. Newer radiation techniques, including intensity modulated radiation therapy, stereotactic body radiation, and proton beam radiation (see Current Research), may help control sarcoma and cause fewer short-term and long-term side effects.

Brachytherapy as outpatient treatment. Doctors are often now able to give brachytherapy as an outpatient procedure. Traditionally, patients stayed in the hospital while the radioactive seeds were in place. This newer procedure uses specialized equipment that can painlessly insert the radiation seeds and remove them after about 15 minutes one or two times a day. This may allow patients to go home during treatment, or if the patient needs to be in the hospital while they are still recovering from the surgery, the patient will not need to be in an isolated, shielded room because the patient will be free of radioactivity for most of the day. This means many patients can return home if medically able or, if still hospitalized, can enjoy visits by family and friends without any concern about radiation exposure.

Learn more about radiation therapy.

Chemotherapy

Chemotherapy is the use of drugs to kill cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Systemic chemotherapy is delivered through the bloodstream to reach cancer cells throughout the body. Chemotherapy is given by a medical oncologist, a doctor who specializes in treating cancer with medication. A chemotherapy regimen usually consists of a specific number of cycles given over a set period of time. Most chemotherapy drugs are given by injection into a vein (called intravenous or IV injection). A patient may receive one drug at a time or combinations of different drugs at the same time.

Different drugs are used to treat different types and subtypes of sarcoma. Chemotherapy for sarcoma can usually be given as an outpatient treatment. Some types of chemotherapy that might be used alone or in combination for STS include:

  • doxorubicin (Adriamycin)
  • epirubicin (Ellence)
  • liposomal doxorubicin (Doxil, Dox-SL, Evacet, LipoDox)
  • ifosfamide (Cyfos, Ifex, Ifosfamidum)
  • gemcitabine (Gemzar)
  • docetaxel (Taxotere)
  • dacarbazine (DTIC-Dome)
  • temozolomide (Methazolastone, Temodar)
  • pazopanib (Votrient)
  • In Europe, trabectedin (Yondelis)

 The specific drugs used to treat sarcoma depends the subtype of sarcoma. Some chemotherapy drugs are only used for certain subtypes of sarcoma.

For GIST:

  • imatinib (Gleevec)
  • sunitinib (Sutent)
  • regorafenib (Stivarga)

For angiosarcoma:

  • paclitaxel (Taxol)
  • docetaxel (Docefrez, Taxotere)
  • sorafenib (Nexavar)
  • bevacizumab (Avastin)

For rhabdomyosarcoma and Ewing sarcoma of soft tissue (or bone):

  • vincristine (Oncovin, Vincasar)
  • etoposide (VePesid, Toposar)
  • actinomycin (Cosmegen, Lyovac Cosmegen)
  • cyclophosphamide (Cytoxan, Clafen, Neosar)
  • topotecan (Hycamptin)
  • irinotecan (Camptosar)

Chemotherapy is often used when a sarcoma has already spread. In addition, certain types of sarcoma might be treated with chemotherapy before surgery to more easily remove the tumor with surgery. Chemotherapy may be given alone or in combination with surgery and/or radiation therapy. Chemotherapy given before surgery may be called by different names, including preoperative chemotherapy, neoadjuvant chemotherapy, or induction chemotherapy.

If a patient has not received chemotherapy before surgery, chemotherapy may be given to destroy any microscopic tumor cells that remain after a patient has recovered from surgery. Chemotherapy given after surgery is called adjuvant chemotherapy or postoperative chemotherapy.

Side effects vary depending on the drug and the dose. Talk with your doctor about the potential side effects, how long they may last, and how they can be relieved. Learn more about chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions by using searchable drug databases.

Targeted therapy/kinase-directed therapy

Targeted therapy is a treatment that targets the cancer’s specific proteins or the tissue environment that contributes to cancer growth and survival, usually by blocking the action of proteins in cells called kinases. This type of treatment blocks the growth and spread of cancer cells while limiting damage to normal cells.

Recent studies show that not all tumors have the same targets (kinases). To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors in your tumor. As a result, doctors can better match each patient with the most effective treatment whenever possible. In addition, many research studies are taking place now to find out more about specific molecular targets and new treatments directed at them (see Current Research).

In 2002, the U.S. Food and Drug Administration (FDA) approved imatinib for the treatment of GIST in advanced stages. This drug is now the standard first-line treatment for GIST worldwide. In 2006, a second targeted therapy, sunitinib, was approved for the treatment of GIST when imatinib doesn’t work. More recently, imatinib has been approved for use for patients with GIST after initial surgery, to try to prevent recurrence in patients who might have a high risk of recurrence. In addition, imatinib is approved for the treatment of patients with advanced stage dermatofibrosarcoma protuberans (DFSP). Trabectedin was approved in 2007 outside of the United States for patients with sarcoma when conventional chemotherapy failed to control the disease. Pazopanib has also been approved for patients with advanced soft tissue sarcoma who have tried chemotherapy. Learn more about targeted treatments.

Palliative/supportive care

Cancer and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care can help a person at any stage of illness. People often receive treatment for the cancer and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Before treatment begins, talk with your health care team about the possible side effects of your specific treatment plan and supportive care options. And during and after treatment, be sure to tell your doctor or another health care team member if you are experiencing a problem, so it is addressed as quickly as possible. Learn more about palliative care.

Recurrent sarcoma

A remission is when cancer cannot be detected in the body and there are no symptoms. This may also be called “no evidence of disease” or NED. 

A remission can be temporary or permanent. This uncertainty leads to many survivors feeling worried or anxious that the cancer will come back. While many remissions are permanent, it’s important to talk with your doctor about the possibility of the cancer returning. Understanding the risk of recurrence and the treatment options may help you feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence.

If the cancer does return after the original treatment, it is called recurrent cancer. A recurrence may start in the tissue where the sarcoma first appeared (local recurrence) or in another place (regional or distant recurrence).

If the sarcoma was originally in the arm or leg, the most common place a recurrence appears is in lungs. Patients treated for sarcoma of the abdomen or trunk are at risk for local, regional, or distant recurrence.

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence. After testing is done, you and your doctor will talk about your treatment options. Often the treatment plan will include the therapies described above (such as surgery, chemotherapy, and radiation therapy) but may be used in a different combination or given at a different pace. Your doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent cancer.

Local recurrences can often be successfully treated with additional surgery, if possible, plus radiation therapy in some patients. Treatment for a distant recurrence is most successful for patients who have a small number of tumors that have spread to the lung that can be completely removed surgically or destroyed with radiofrequency ablation (use of a needle inserted into the tumor to destroy the cancer with an electrical current) or focused, high-dose radiation therapy.

People who have been treated for sarcoma should have regular follow-up examinations to look for a possible recurrence based on a schedule set up by their oncologist (see After Treatment).

People with recurrent cancer often experience emotions such as disbelief or fear. Patients are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. Learn more about dealing with cancer recurrence.

Metastatic sarcoma

If cancer has spread to another location in the body, it is called metastatic cancer. Patients with this diagnosis are encouraged to talk with doctors who are experienced in treating this stage of cancer, because there can be different opinions about the best treatment plan. Learn more about seeking a second opinion before starting treatment, so you are comfortable with the treatment plan chosen. This discussion may include clinical trials.

Your health care team may recommend a treatment plan that includes surgery alone, surgery plus radiation therapy, surgery plus chemotherapy, or chemotherapy alone. Rarely, when the tumor is not growing, a “watch and wait” (also called active surveillance) approach may be used. This means that the patient is closely monitored and active treatment begins only if the tumor begins to grow. Supportive care will also be important to help relieve symptoms and side effects.

Rarely, for patients with a very large tumor involving the major nerves and blood vessels of the arm or leg, amputation (surgical removal of the limb) is necessary to control the tumor. This can also be necessary if the tumor grows back in the arm or leg after prior surgery, radiation therapy, and/or chemotherapy. It’s important to remember that the operation that results in the most useful and strongest limb may be different from the one that gives the most normal appearance. If amputation is needed, rehabilitation, including physical therapy, can help maximize the patient’s physical functioning. Rehabilitation can also help a person cope with the social and emotional effects of losing a limb.

If treatment fails

Recovery from cancer is not always possible. If treatment is not successful, the disease may be called advanced or terminal cancer.

This diagnosis is stressful, and this is difficult to discuss for many people. However, it is important to have open and honest conversations with your doctor and health care team to express your feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families. Making sure a person is physically comfortable and free from pain is extremely important.

Palliative care given toward the end of a person’s life is called hospice care. You and your family are encouraged to think about where you would be most comfortable: at home, in the hospital, or in a hospice environment. Nursing care and special equipment can make staying at home a workable alternative for many families. Learn more about advanced cancer care planning.

After the death of a loved one, many people need support to help cope with the loss. Learn more about grief and bereavement.

Find out more about common terms used during cancer treatment.

The next section helps explain clinical trials, which are research studies. Use the menu on the side of your screen to select About Clinical Trials, or you can select another section, to continue reading this guide.  

Sarcoma - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2013

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. To see other pages, use the menu on the side of your screen.

Doctors and scientists are always looking for better ways to treat patients with sarcoma. To make scientific advances, doctors create research studies involving people, called clinical trials.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Patients who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and manage the late effects that may occur after treatment. Talk with your doctor about clinical trials regarding side effects. In addition, there are ongoing studies about ways to prevent the disease.

Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that these studies are the only way to make progress in treating sarcoma. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with sarcoma.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

To join a clinical trial, patients must participate in a process known as informed consent. During informed consent, the doctor should list all of the patient’s options so that the person understands how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different from the risks of standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.

For specific topics being studied for sarcoma, learn more in the Current Research section.

Patients who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that patients participating in a clinical trial talk with their doctor and researchers about who will be providing their treatment and care during the clinical trial, after the clinical trial ends, and/or if the patient chooses to leave the clinical trial before it ends.

The next section helps explain the areas of research going on today about this type of cancer. Use the menu on the side of your screen to select Latest Research, or you can select another section, to continue reading this guide.  

Sarcoma - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2013

ON THIS PAGE: You will read about the scientific research being done now to learn more about this type of cancer and how to treat it. To see other pages, use the menu on the side of your screen.

Doctors are working to learn more about sarcoma, ways to prevent it, how to best treat it, and how to provide the best care to people diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your doctor about the diagnostic and treatment options best for you.

Proton or particle beam radiation therapy. Proton therapy (also called proton beam therapy) is a type of radiation treatment that uses protons rather than x-rays. At high energy, protons can destroy cancer cells. Protons travel only the desired distance in tissue, allowing doctors to target the radiation much more precisely at the tumor and deliver a smaller dose to surrounding healthy tissue. This procedure is currently only available in a few specialized cancer centers in the United States and is most commonly used in areas of the body where there are vital organs or structures nearby, such as the brain or spinal cord. Learn more about proton therapy. Radiation treatment with heavier charged particles, known as carbon ions, are being used and studied for the treatment of sarcomas in Japan and Germany.

Improved drug delivery. Some chemotherapy drugs are incorporated into fat molecules called liposomes to improve the absorption and distribution of the drug in the patient’s body.

New chemotherapy drugs. New chemotherapy is being developed and tested that may be effective in treating some subtypes of STS. An example is trabectedin (Yondelis), which has been approved in Europe. Trabectedin has been shown to have a very high level of activity in the treatment of a specific subtype of liposarcoma called myxoid-round cell liposarcoma. However, in the United States, FDA only allows patients access to it under special circumstances. Eribulin (Halaven), a drug approved for treatment of breast cancer, has shown promising results in an early trial in Europe and is now being investigated in a large, international study. Researchers are also working to find versions of standard chemotherapy to treat sarcoma that cause fewer side effects. An example is three new versions of ifosfamide—called palifosfamide, glufosfamide, and TH-302—that are being studied in ongoing clinical trials. Learn more about drug development and approval.

Targeted therapy. As explained in the Treatment section, imatinib and sunitinib, types of drugs called tyrosine kinase inhibitors, are the first targeted (kinase-specific) therapies approved to treat specific types of sarcoma, most commonly GIST. Pazopanib (Votrient) was recently approved for use in sarcomas other than liposarcoma and GIST in situations where other chemotherapy is not working. Currently, researchers are identifying new kinase inhibitors to use for an individual patient and the order that the drugs should be used in a patient’s treatment plan.

Regorafenib is a new targeted therapy with significant activity in patients with advanced GIST who have already been treated with imatinib and suntinib. The FDA is currently reviewing a phase III clinical trial of this drug.

Other clinical trials are looking at a type of targeted therapy called anti-angiogenesis therapy. It is focused on preventing the formation of new blood vessels in order to starve the tumor of the oxygen and nutrients it needs to grow.

Targeted oncogene treatments. Drugs are being researched that may block one or more of the proteins found in tumor cells that help the tumor grow and spread.

Genetics. Researchers are learning that some sarcomas have unique genetic “fingerprints.” Understanding these fingerprints may help doctors determine better treatments and possibly better predict a patient’s prognosis.

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current sarcoma treatments in order to improve patients’ comfort and quality of life.

Learn more about common statistical terms used in cancer research.

To find clinical trials specific to your diagnosis, talk with your doctor or search online clinical trial databases now.

The next section addresses how to cope with the symptoms of the disease or the side effects of its treatment. Use the menu on the side of your screen to select Coping with Side Effects, or you can select another section, to continue reading this guide.  

Sarcoma - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2013

ON THIS PAGE: You will find out more about steps to take to help cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu on the side of your screen.

Fear of treatment side effects is common after a diagnosis of cancer, but it may help to know that preventing and controlling side effects is a major focus of your health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects of each treatment option for sarcoma are described within the Treatment section. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the cancer’s stage, the length and dosage of treatment(s), and your overall health.

Before treatment begins, talk with your doctor about possible side effects of each type of treatment you will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving you may need during treatment and recovery, as family members and friends often play an important role in the care of a person with sarcoma. Learn more about caregiving.

In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies. Learn more about the importance of addressing such needs, including concerns about managing the cost of your cancer care

During and after treatment, be sure to tell the health care team about the side effects you experience, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care. Learn more by reading the After Treatment section or talking with your doctor.

The next section helps explain medical tests and check-ups needed after finishing cancer treatment. Use the menu on the side of your screen to select After Treatment, or you can select another section, to continue reading this guide.  

Sarcoma - After Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2013

ON THIS PAGE: You will read about your medical care after cancer treatment is finished and why this follow-up care is important. To see other pages, use the menu on the side of your screen.

After treatment for sarcoma ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years. ASCO offers cancer treatment summary forms to help keep track of the cancer treatment you received and develop a survivorship care plan once treatment is completed.

Regularly scheduled follow-up visits with the doctors involved in your treatment are important to find any possible tumor recurrence and to help manage and hopefully prevent some side effects related to treatment. A common follow-up schedule includes visits every three to four months for the first three years after treatment, visits every six months until five years after treatment, and annual visits thereafter. Chest x-rays or CT scans will be done regularly to look for any possible spread of cancer to the lungs during these follow-up visits. Imaging tests, such as MRI, ultrasound, CT scan, and/or PET scan are also sometimes performed on the area where the tumor began.

People who have been treated for sarcoma should be particularly careful to talk with their doctor about any new symptoms, such as a cough, pain, or a new lump. It may be a sign of a cancer recurrence, a late effect of treatment, or a problem unrelated to cancer. If a recurrence happens, it is likely to be within the first two years, but some sarcomas can recur much later.

For people who received radiation therapy, the region of the body that received radiation therapy can be at risk for limb swelling (lymphedema), fracture of the thigh or leg bones, poor mobility of joints, and fibrosis (hardness) of the soft tissues. Rarely, another sarcoma that is different from the original tumor may develop as a result of radiation therapy. Limb swelling can be managed with compression stockings and other special therapies that can be prescribed by doctors. Bone fractures may be prevented by avoiding certain high-impact exercises, which patients should talk about with their doctors. Joint mobility can be improved with a rehabilitation program. Fibrosis may lessened with several months of treatment with a combination of vitamin E and pentoxifylline, another oral medication. Skin that received radiation therapy should be regularly protected from sun exposure with clothing or sunscreen to reduce the chance of skin cancer developing in that area.

For patients treated for sarcoma in an arm or leg, a rehabilitation program after surgery or radiation therapy can help the patient regain or maintain limb function. Range-of-motion exercises, strengthening exercises, and a program to reduce limb swelling, if needed, may be recommended. Visiting a rehabilitation medicine specialist may be extremely important to help the patient receive the most appropriate rehabilitation after treatment. The majority of patients with a sarcoma in an arm or leg can be successfully treated and do maintain good limb function. However, particularly when the treatment included amputation, prosthetic services (artificial limbs) and mental health support can help manage the adjustment to life following treatment. Learn more about rehabilitation.

People recovering from sarcoma are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. Moderate physical activity can help rebuild your strength and energy level. Your doctor can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about the next steps to take in survivorship, including making positive lifestyle changes.

Find out more about common terms used after cancer treatment is complete.

The next section offers a list of questions you may want to ask. Use the menu on the side of your screen to select Questions to Ask the Doctor, or you can select another section, to continue reading this guide.  

Sarcoma - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2013

ON THIS PAGE: You will find some questions to ask your doctor or other members of your health care team, to help you better understand your diagnosis, treatment plan, and overall care. To see other pages, use the menu on the side of your screen.

Talking often with the doctor is important to make informed decisions about your health care. These suggested questions are a starting point to help you learn more about your cancer care and treatment. You are also encouraged to ask additional questions that are important to you.

General questions:

  • What type of sarcoma has been diagnosed?
  • Has this diagnosis been reviewed by a pathologist who is experienced in the diagnosis and classification of sarcoma?
  • What does my diagnosis mean?
  • Where is the tumor located?
  • What is the sarcoma’s stage and grade? What does this mean?
  • Can you explain my pathology report (laboratory test results) to me?
  • Should I see a sarcoma specialist?
  • Was my case discussed in a multidisciplinary conference? What does this mean?
  • What are my treatment options?
  • What clinical trials are open to me?
  • Who will be part of my health care team, and what does each member do?
  • Who will be coordinating my overall treatment and follow-up care?
  • What treatment plan do you recommend? Why?
  • What is the goal of each treatment?
  • What are the possible side effects of this treatment, both in the short term and the long term?
  • How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?
  • Could this treatment affect my sex life? If so, how and for how long?
  • Could this treatment affect my ability to become pregnant or have children? If so, should I talk with a fertility specialist before cancer treatment begins?
  • If I’m worried about managing the costs related to my cancer care, who can help me with these concerns?
  • What support services are available to me? To my family?
  • Whom do I call for questions or problems?

Questions to ask before surgery:

  • Should I have a biopsy before surgery?
  • What will happen during this surgery?
  • Will the surgery be performed in en-bloc resection? What does this mean?
  • How experienced is this surgeon with sarcoma? How many sarcoma operations has he or she done in the past year?
  • What are the possible side effects of my surgery, both in the short term and long term?
  • Will I need to stay in the hospital for this surgery? For how long?
  • Does the stage of cancer affect the decision for surgery? How?
  • Do I need chemotherapy and/or radiation before the surgery? After the surgery?
  • Will I need rehabilitative services after the surgery?

Questions to ask before radiation therapy:

  • What type of radiation therapy is recommended?
  • How experienced is the radiation oncologist with sarcoma? How many people with sarcoma has he or she treated in the past year?
  • How many treatments will I need, and how long will each one take?
  • What side effects can I expect from this treatment?
  • What can be done to prevent or to treat the side effects?

Questions to ask before chemotherapy or targeted therapy:

  • What type of medication is recommended?
  • How experienced is the medical oncologist with sarcoma? How many people with sarcoma has he or she treated in the past year?
  • Are there other chemotherapy or targeted treatments available?
  • Should I consider a clinical trial?
  • How many treatments will I need, and how long will each take?
  • What side effects can I expect from this treatment?
  • What can be done to prevent or manage these side effects?

After treatment:

  • What are the chances that the sarcoma will return?
  • What follow-up tests do I need, and how often do I need them?

The next section offers some more resources that may be helpful to you. Use the menu on the side of your screen to select Additional Resources, or you can select another section, to continue reading this guide.  

Sarcoma - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 01/2013

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about cancer care and treatment. This is the final page of Cancer.Net’s Guide to Sarcoma. To go back and review other pages, use the menu on the side of your screen.

In addition to Cancer.Net, there are other sources of information about this type of cancer available online. Cancer.Net maintains a list of national, not-for-profit organizations that may be helpful in finding additional information, services, and support. As always, be sure to talk with your doctor about questions you may have about information you find about this disease.

View organizations that offer information on this specific type of cancer.

This is the end of Cancer.Net’s Guide to Sarcoma. Use the menu on the side of your screen to select another section, to continue reading this guide.