View All Pages

Sarcoma, Soft Tissue - Introduction

Approved by the Cancer.Net Editorial Board, 05/2019

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 Soft-Tissue Sarcoma. Use the menu to see other pages. Think of that menu as a roadmap for this complete guide.

Cancer begins when healthy cells change and grow out of control, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can grow and spread to other parts of the body. A benign tumor means the tumor can grow but will not spread.

About sarcomas

Sarcomas can start in any part of the body, such as the bone or soft tissue.

  • 60% begin in an arm or leg

  • 30% start in the torso or abdomen

  • 10% occur in the head or neck

Both children and adults can develop a sarcoma. It is rare, accounting for about 1% of all adult cancers. However, sarcomas represent about 15% of all childhood cancers.

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

About soft-tissue sarcoma

Soft-tissue sarcoma (STS) is a group of cancers that begin in the connective tissues that support and connect the body, including:

  • Blood vessels

  • Fat cells

  • Lining of joints

  • Lymph vessels

  • Muscle

  • Nerves

  • Tendons

As a result, STS can occur almost anywhere in the body. When an STS begins and is small, it can go unnoticed because it usually does not cause problems, such as pain. However, as an STS grows, it can interfere with the body's normal functions.

Not all sarcomas are the same. Because there are more than 50 different types of STS and each has a different natural history, meaning where they start, how they affect the body, how fast they grow, and how they respond to treatment, it is more accurate to describe them as a family of related diseases rather than as a single disease. Specific types of sarcoma are often named according to the normal tissue cells they most closely resemble, as listed below. This is different from most other types of cancer, which usually are 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 or other primitive cells. Stem cells are special cells that can mature into specific tissues or organs.

The list below describes several common types of STS and related connective tissue tumors.

Name of Sarcoma

Related Normal Tissue Type

Angiosarcoma

Blood or lymph vessels

Desmoid tumor, also called deep fibromatosis

Fibroblasts, which are the most common type of cells in connective tissue

Ewing family of tumors

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

Fibrosarcoma

Fibroblasts, which are the most common type of cells in connective tissue

Gastrointestinal stromal tumor (GIST)

Specialized neuromuscular cells of the digestive tract

Kaposi sarcoma

Blood vessels

Leiomyosarcoma

Smooth muscle

Liposarcoma

Fat tissue

Myxofibrosarcoma

Connective tissue

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

Cells that wrap around nerve endings, similar to the way insulation wraps around a wire

Rhabdomyosarcoma

Skeletal muscle

Synovial sarcoma

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

Undifferentiated pleomorphic sarcoma (UPS), often referred to as malignant fibrous histiocytoma (MFH) in the past

No obvious related normal tissue; may be a tumor of stem cells or a distant relative of rhabdomyosarcoma

When a tumor is found and the doctor believes it could be sarcoma, it is important to find out the type of sarcoma as part of the diagnosis process. Because there are so many different types of sarcomas, it is best if an expert pathologist who specializes in sarcoma examines the tumor sample. A pathologist is a doctor who uses a microscope to look at the tumor specimen to make the diagnosis. The pathologist will look under the microscope to see the size and shape (called morphology) of the tumor cells and do special stains (called immunohistochemistry) to determine what type of sarcoma it is (this is called the histology). Sometimes they need more information and additional genetic tests are performed, which take longer for the results to come back. Pathologists are looking for new ways to quickly determine a tumor's type because this helps the health care team decide on the treatment plan.

Pathologists also describe a sarcoma by its “grade,” which describes how closely cancer cells look or don’t look like healthy cells when viewed under a microscope. A higher-grade tumor has cells that look more abnormal and are potentially more aggressive. The grade can help the doctor predict how quickly the sarcoma will grow and spread. In general, the lower the tumor’s grade, the less the likelihood it will recur and/or grow. A pathologist with expertise in sarcomas should be involved when sarcoma is suspected. Learn more about grade in the Stages and Grades section.

Looking for More of an Introduction?

If you would like more of an introduction, explore these related items. Please note that these links will take you to other sections on Cancer.Net:

The next section in this guide is Statistics. It helps explain the number of people who are diagnosed with sarcoma and general survival rates. Use the menu to choose a different section to read in this guide.

Sarcoma, Soft Tissue - Statistics

Approved by the Cancer.Net Editorial Board, 05/2019

ON THIS PAGE: You will find information about the number of people who are diagnosed with STS each year. You will also read general information on surviving the disease. Remember, survival rates depend on several factors. Use the menu to see other pages.

This year, about 12,750 people (7,240 men and 5,510 women) will be diagnosed with STS in the United States.

An estimated 5,270 adults and children (2,840 males and 2,430 females) are expected to die of the disease this year.

Sarcomas are described as being localized, locally advanced, or metastatic when they are first found.

  • Localized means the tumor is only in 1 area of the body.

  • Locally advanced means the tumor involves or attaches to nearby tissues or organs. This often means it is not possible to remove the tumor because of its location, size, or the organs it involves.

  • Metastatic means the sarcoma has spread to parts of the body far away from where the sarcoma started.

When the sarcoma starts in an arm or a leg, the 5-year survival rates are slightly higher for each stage when compared with sarcoma that starts in other locations.

This section includes information on how often sarcomas show up as localized, locally advanced, or metastatic. In addition, there is information on survival rates for each situation. This information applies for sarcomas in general and may not apply to a specific type of sarcoma. Talk with your health care team for more information regarding your specific diagnosis.

In general, cancer statistics often include the 5-year survival rate for a type of cancer. The 5-year survival rate tells you what percent of people live at least 5 years after the cancer is found. Percent means how many out of 100. The overall 5-year survival rate for sarcoma is about 65%.

  • About 58% of sarcomas are found as a localized sarcoma. The 5-year survival rate for people with localized sarcoma is 81%.

  • About 18% of sarcomas are found in a locally advanced stage. The 5-year survival rate for people with locally advanced sarcoma is 58%.

  • About 14% of sarcomas are found in a metastatic stage. The 5-year survival rate for people with metastatic sarcoma is 16%.

It is important to note that the length of time a person lives with a sarcoma depends on many factors, including the type of sarcoma, the size, where it is located, and how quickly the tumor cells are growing and dividing, called the grade. If the sarcoma is found at an early stage and has not spread from where it started, surgical treatment is often very effective and many people are cured. However, if the sarcoma has spread to other parts of the body, treatment can usually control the tumor, but it is often incurable. Learn more in the Stages and Grades section.

It is important to remember that statistics on the survival rates for people with STS are an estimate. The estimate comes from annual data based on the number of people with this cancer in the United States. Also, experts measure the survival statistics every 5 years. So the estimate may not show the results of better diagnosis or treatment available for less than 5 years. Talk with your doctor if you have any questions about this information. Learn more about understanding statistics.

Statistics adapted from the American Cancer Society's (ACS) publication, Cancer Facts & Figures 2017: Special Section – Rare Cancers in Adults, and the ACS website (January 2019).

The next section in this guide is Medical Illustrations. It offers drawings of body parts often affected by STS. Use the menu to choose a different section to read in this guide.

Sarcoma, Soft Tissue - Medical Illustrations

Approved by the Cancer.Net Editorial Board, 05/2019

ON THIS PAGE: You will find a drawing of the main body parts affected by sarcoma. Use the menu to see other pages.

This illustration shows areas in the body in which soft-tissue sarcomas are likely to occur. In the head and neck area, this includes the nasal cavity, which is the air filled cavity behind the nose; the nasopharynx, the air passageway at the upper part of the throat behind the nose; and the soft palate, at the back of the mouth just in front the nasopharynx. The illustration also highlights the skeleton, abdomen, and extremities (arms and legs). A sagittal cross-section of a male pelvis shows the peritoneum, the membrane that lines the abdominal cavity and organs; the rectus abdominis muscle, which runs down the front of the abdomen; and the fatty tissue that covers the rectus abdominis muscle. Copyright 2004 American Society of Clinical Oncology. Robert Morreale/Visual Explanations, LLC.

The next section in this guide is Risk FactorsIt explains the factors that may increase the chance of developing sarcoma. Use the menu to choose a different section to read in this guide.

Sarcoma, Soft Tissue - Risk Factors

Approved by the Cancer.Net Editorial Board, 05/2019

ON THIS PAGE: You will find out more about the factors that increase the chance of developing sarcoma. Use the menu to see other pages.

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 wit h several risk factors never develop cancer, while others with no known risk factors do. 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 may raise a person’s risk of developing sarcoma:

  • Previous radiation therapy. People who were treated with radiation therapy for a previous cancer, usually more than 5 years ago, have a slightly increased risk of later developing sarcoma.

  • Genetics. People with certain inherited diseases have a higher risk of sarcoma. These diseases include neurofibromatosis type 1, also known as von Recklinghausen’s disease; familial adenomatous polyposis, also known as Gardner syndrome; Werner syndrome; tuberous sclerosis complex; nevoid basal cell carcinoma syndrome; Li-Fraumeni syndrome; and retinoblastoma.

  • Immune system abnormalities. People with problems with their immune system have a higher risk of several types of cancer, whether from infections such as human immunodeficiency virus (HIV), cancers such as chronic lymphocytic leukemia, or autoimmune conditions such as lupus or psoriasis.

  • Lymphedema. People who have surgery or radiation therapy for other reasons may have swelling of an arm, leg, or other part of the body as a side effect of the treatment. This is called lymphedema. For example, lymphedema is common after breast surgery when lymph nodes are removed from the armpit area. People can also be born with lymphedema. Sarcomas, such as angiosarcoma, occasionally form in areas where lymphedema had developed.

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

The next section in this guide is Symptoms and Signs. It explains what body changes or medical problems sarcoma can cause. Use the menu to choose a different section to read in this guide.

Sarcoma, Soft Tissue - Symptoms and Signs

Approved by the Cancer.Net Editorial Board, 05/2019

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. Use the menu to see other pages.

Sarcomas can start in any part of the body. The types of symptoms that people have from a sarcoma depend on where it starts. People with sarcoma may experience the following symptoms or signs. Sometimes, people with sarcoma do not have any of these changes. Or, the cause of a symptom may be a different medical condition that is not cancer.

STS rarely causes symptoms in the early stages. The first sign of a sarcoma in an arm, leg, or torso may be a painless lump or swelling. Most lumps are not sarcoma. The most common soft-tissue lumps are lipomas. Lipomas are made of fat cells and are not cancer. Lipomas have often been there for many years and rarely change in size. In the uterus, benign tumors called fibroids, or leiomyomas, far outnumber sarcomas.

However, it’s important to talk with your doctor about any lumps that are larger than 2 inches (5 centimeters), grow larger, or are painful, regardless of their location. People with a sarcoma that starts in the abdomen may not have any symptoms, or they may have pain or a sense of fullness.

Because sarcoma can develop in flexible, elastic tissues or deep spaces in the body, the tumor can often 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.

Sarcomas that arise in other parts of the body may cause other symptoms or signs. For example, sarcomas that arise in the uterus can cause uterine bleeding or an increase in the size of the uterus. Tumors that start in the heart or a lung may cause breathing problems or pain in the chest.

If you are concerned about any changes you experience, please talk with your doctor. Your doctor will ask how long and how often you’ve been experiencing the symptom(s), in addition to other questions. This is to help figure out the cause of the problem, called a diagnosis.

If a sarcoma is diagnosed, relieving symptoms remains an important part of cancer care and treatment. This may be called palliative care or supportive care. It is often started soon after diagnosis and continued throughout treatment. Be sure to talk with your health care team about the symptoms you experience, including any new symptoms or a change in symptoms.

The next section in this guide is Diagnosis. It explains what tests may be needed to learn more about the cause of the symptoms. Use the menu to choose a different section to read in this guide.

Sarcoma, Soft Tissue - Diagnosis

Approved by the Cancer.Net Editorial Board, 05/2019

ON THIS PAGE: You will find a list of common tests, procedures, and scans that doctors use to find the cause of a medical problem. Use the menu to see other pages.

Doctors use many tests to find, or diagnose, cancer. They also do tests to learn if cancer has spread to another part of the body from where it started. If this happens, it is called metastasis. For example, imaging tests can show if the cancer has spread. Imaging tests show pictures of the inside of the body. Doctors may also do tests to learn which treatments may work best.

For most types of cancer, a biopsy is the only sure way for the doctor to know if an area of the body has cancer. In a biopsy, the doctor takes a small sample of tissue for testing in a laboratory. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Although biopsies have a small chance of not giving a definite answer, they are very important to allow your doctor make a clear diagnosis and develop a clear team-based treatment plan.

This section describes options for diagnosing sarcoma. Not all tests listed below will be used for every person. Your doctor may consider these factors when choosing a diagnostic test:

  • The type of cancer suspected

  • Your signs and symptoms

  • Your age and general health

  • The results of earlier medical tests

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. Sarcomas are rare. If a sarcoma is suspected, it is very important to talk with a doctor who has experience with this type of cancer.

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, some of the tests described below may be used to diagnose sarcoma.

Imaging tests

Both benign and cancerous tumors can show up on imaging tests, such as an x-ray. Radiologists will use the way the tumor looks on the test to help determine whether it may be benign or cancerous; however, a biopsy is almost always needed.

  • 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. X-ray is particularly useful for bone sarcomas, but less valuable for STS.

  • Ultrasound. An ultrasound uses sound waves to create a picture and may be used to look at lumps under the skin or other organs in the body.

  • Computed tomography (CT or CAT) scan. A CT scan takes pictures of the inside of the body using x-rays taken from different angles. A computer combines these pictures into a detailed, 3-dimensional image that shows any abnormalities or tumors. A CT scan can be used to measure the tumor’s size or see if the cancer has spread somewhere else. Sometimes, a special dye called contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein and/or be a pill or liquid the patient must drink.

  • Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. MRI can be used to measure the tumor’s size. A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein. MRI is often used to determine whether a sarcoma can be removed with surgery.

  • Positron emission tomography (PET) or PET-CT scan. A PET scan is usually combined with a CT scan (see above), called a PET-CT scan. However, you may hear your doctor refer to this procedure just as a PET scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive sugar substance is injected into the patient’s body. This sugar substance is taken up by cells 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 inside of the body. This technique can be used to look at both the tumor’s structure and how much energy is used by the tumor and normal tissues. This information can be helpful in planning treatment and evaluating how well treatment is working, but it is usually not performed in all cases of known or suspected STS.

Biopsy and tissue tests

Imaging tests may suggest the diagnosis of sarcoma, but a biopsy will be needed to confirm the diagnosis and to find out the type of sarcoma. 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 reliable diagnosis. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease.

    Because STS is uncommon, an expert pathologist should review the tissue sample to properly diagnose a sarcoma. Sometimes properly diagnosing a sarcoma requires special tests on the tumor tissue, and it is best if a specialist who sees this type of cancer regularly does this.

    There are different types of biopsies. For a needle biopsy, a doctor removes a small sample of tissue from the tumor with a needle-like instrument—usually a core needle biopsy. This may be performed with the help of ultrasound, CT scan, or MRI to precisely guide the needle into the tumor. In an incisional biopsy, a surgeon cuts into the tumor and removes a sample of tissue. In an excisional biopsy, the surgeon removes the entire tumor. Excisional biopsies are generally not recommended for sarcomas because second surgeries are often needed to completely remove the tumor.

    The type of biopsy and how it is done are important in diagnosing and treating sarcomas, so patients should be seen in a sarcoma specialty center even before the biopsy is performed. Because STS tumors are uncommon and there are many different types of STS, it is important to have an expert pathologist review the sample of tissue removed to appropriately diagnose a sarcoma. People who doctors suspect have sarcoma should be sent to a sarcoma center, ideally before a biopsy is done, so that the treating surgeon can identify the location for the biopsy.

  • Tissue testing of the tumor. Your doctor or the pathologist looking at the sarcoma 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 what the treatment should be, because each sarcoma can be as different from one another as breast cancer is different from colon cancer (see Types of Treatment).

After 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 and grading.

The next section in this guide is Stages and Grades. It explains the system doctors use to describe the extent of the disease. Use the menu to choose a different section to read in this guide.

Sarcoma, Soft Tissue - Stages and Grades

Approved by the Cancer.Net Editorial Board, 05/2019

ON THIS PAGE: You will learn about how doctors describe a cancer’s growth or spread, and how quickly it may grow. This is called the stage and grade. Use the menu to see other pages.

Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting other parts of the body.

Doctors use diagnostic tests to find out the cancer's stage, so staging may not be complete until all of 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, which is the chance of recovery. There are different stage descriptions for different types of cancer.

There are several systems used to describe the stages of sarcomas. These systems are somewhat different from each other, but all use an overall stage to help predict a person’s prognosis.

TNM staging system

One tool that doctors use to describe the stage is the TNM system. Doctors use the results from diagnostic tests and scans to answer these questions:

  • Tumor (T): How large is the primary tumor? Where is it located?

  • Node (N): Has the tumor spread to the lymph nodes? If so, where and how many?

  • Metastasis (M): Has the cancer spread to other parts of the body? If so, where and how much?

The results are combined to determine the stage of cancer for each person. There are 4 stages: stages I through IV (1 through 4). The stage provides a common way of describing the cancer, so doctors can work together to plan the best treatments.

Here are more details on each part of the TNM system for sarcomas. The staging systems are different depending on the location of the sarcoma. This section describes:

  • STS in the trunk and extremities, which generally includes all of the body from the head down.

  • STS in the retroperitoneum, which is roughly defined as the abdominal cavity.

For TNM staging information for STS found in other parts of the body, visit the website of the American Joint Committee on Cancer. Please note that this link takes you to a separate, independent website.

Tumor (T)

Using the TNM system, the "T" plus a number is used to describe the size of the tumor. Tumor size is measured in centimeters (cm). A centimeter is roughly equal to the width of a standard pen or pencil.

TX: The primary tumor cannot be evaluated.

T0 (T plus zero): There is no evidence of a primary tumor.

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

T2: The tumor is larger than 5 cm but not larger than 10 cm.

T3: The tumor is larger than 10 cm but not larger than 15 cm.

T4: The tumor is larger than 15 cm.

Node (N)

The "N" in the TNM staging system stands for lymph nodes. These tiny, bean-shaped organs help fight infection. 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. STS usually does not spread to lymph nodes, and removing lymph nodes is not typically recommended for most types of sarcomas.

N0 (N plus zero): The cancer has not spread to regional lymph nodes.

N1: The cancer has spread to regional lymph nodes.

Metastasis (M)

The "M" in the TNM system describes whether the cancer has spread to other parts of the body, called distant metastasis.

M0 (M plus zero): The cancer has not metastasized.

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

Grade (G)

Doctors also describe this type of cancer by its grade (G). The grade describes how much cancer cells look like healthy cells when viewed under a microscope.

The doctor compares the cancerous tissue with healthy tissue. Healthy tissue usually contains many different types of cells grouped together. If the cancer looks similar to healthy tissue and has different cell groupings, it is called “differentiated” or a “low-grade tumor.” If the cancerous tissue looks very different from healthy tissue, it is called “poorly differentiated” or a “high-grade tumor.” The cancer’s grade may help the doctor predict how quickly the cancer will spread. In general, the lower the tumor’s grade, the better the prognosis.

There are 4 grades for sarcoma: GX (the grade cannot be evaluated), G1, G2, and G3. The grades are determined based on the factors below:

  • Differentiation: How different the cancer cells are from normal tissue cells.

  • Mitotic count: How many tumor cells are dividing.

  • Tumor necrosis: How much of the tumor has cells that are dying.

The lower the combined score for these 3 factors, the lower the grade. A lower grade means that the tumor is less aggressive and that a patient’s prognosis is better. This is the recommended grading system from the French Federation of Cancer Centers Sarcoma Group (French Fédération Nationale des Centres de Lutte Contre le Cancer or FNCLCC), but other systems 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, M, and G classifications.

Because sarcomas can arise in any part of the body, there are different stage groupings for different sarcomas. It is best to discuss your pathology results and stage with your doctor. General stage groupings for STS are defined below:

Stage I: The tumor is small and low grade (GX or G1).

Stage II: The tumor is small and higher grade (G2 or G3).

Stage III: The tumor is larger and higher grade (G2 or G3).

Stage IV: The cancer has spread to other parts of the body. The original tumor can be any size, any grade, and may or may not have spread to the regional lymph nodes (any G).

This staging system applies to most types of STS, except Kaposi sarcoma; GIST; infantile fibrosarcoma; sarcomas that begin in the central nervous system, which includes 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 the cancer does return, there will be another round of tests to learn about the extent of the recurrence. These tests and scans are often similar to those done at the time of the original diagnosis.

Used with permission of the American College of Surgeons, Chicago, Illinois. The original and primary source for this information is the AJCC Cancer Staging Manual, Eighth Edition (2017), published by Springer International Publishing.

Information about the cancer’s stage will help the doctor recommend a specific treatment plan. The next section in this guide is Types of Treatment. Use the menu to choose a different section to read in this guide.

Sarcoma, Soft Tissue - Types of Treatment

Approved by the Cancer.Net Editorial Board, 05/2019

ON THIS PAGE: You will learn about the different types of treatments doctors use for people with sarcoma. Use the menu to see other pages.

This section explains the types of treatments that are the standard of care for sarcoma. “Standard of care” means the best treatments known. When making treatment plan decisions, you are encouraged to consider clinical trials as an option. A clinical trial is a research study that tests a new approach to treatment. Doctors want to learn whether the new treatment is safe, effective, and possibly better than the standard treatment. Clinical trials can test a new drug, a new combination of standard treatments, or new doses of standard drugs or other treatments. Clinical trials are an option to consider for treatment and care for all stages of cancer. Your doctor can help you consider all your treatment options. Learn more about clinical trials in the About Clinical Trials and Latest Research sections of this guide.

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. Cancer care teams include a variety of other health care professionals, such as physician assistants, oncology nurses, social workers, pharmacists, counselors, dietitians, and others.

Descriptions of the common types of treatments used for sarcomas are listed below. Your care plan may also include treatment for symptoms and side effects, an important part of cancer care.

Treatment options and recommendations depend on several factors, including the type, stage, and grade of sarcoma, possible side effects, and the patient’s preferences and overall health. Take time to learn about all of your treatment options and be sure to ask questions about things that are unclear. Talk with your doctor about the goals of each treatment and what you can expect while receiving the treatment. These types of talks are called “shared decision making.” Shared decision making is when you and your doctors work together to choose treatments that fit the goals of your care. Shared decision making is particularly important for sarcomas because there are different treatment options. Learn more about making treatment decisions.

Surgery

Surgery is the removal of the tumor and some surrounding healthy tissue during an operation. Before surgery, it’s important to have a biopsy and appropriate imaging to confirm the diagnosis (see Diagnosis). After a biopsy, surgery is typically an important part of the treatment plan if the tumor is localized (located in only 1 area). Surgical oncologists and orthopedic oncologists are doctors who specialize in treating sarcomas using surgery.

The surgeon's goal is to remove the tumor and enough normal tissue surrounding it to obtain a clean margin around the tumor. A “clean margin” means there are no tumor cells visible at the borders of the surgical specimen. This is currently the best method available to ensure that there are no tumor cells left in the area from which the tumor was removed. Small low-grade sarcomas can usually be effectively removed by surgery alone. Those that are high grade and larger than 2 inches (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. They also may be used during and after surgery to destroy any remaining cancer cells.

Rarely, for patients with a very large tumor involving the major nerves and blood vessels of the arm or leg, surgical removal of the limb, called amputation, is required to control the tumor. This can also be necessary if the tumor grows back in the arm or leg after surgery, radiation therapy, and/or chemotherapy have been completed. 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 physical function. Rehabilitation can also help a person cope with the social and emotional effects of losing a limb. People who have had an amputation can often wear a prosthesis, depending on the type of amputation.

Before surgery, talk with your health care team about the possible side effects from the specific surgery you will have, including the recovery period. Learn more about the basics of cancer surgery.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. Since sarcomas are rare, it is very important to talk with a radiation oncologist who has experience treating sarcoma.

A radiation therapy regimen, or 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 that it may be 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 critical structures in the arm or leg if the sarcoma is located in one of those places.

Radiation therapy can damage normal cells, but because it is focused around the tumor, side effects are usually limited to those areas.

External-beam radiation therapy

The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body.

The way external-beam radiation is used has changed over the past 20 years. It is now possible to give many small beams of radiation that turn on and off as the radiation machine rotates around the body. This is called intensity modulated radiation therapy (IMRT) and is now typically used for sarcomas. IMRT focuses more radiation on the tumor site and less on the normal tissues. As a result, there are fewer side effects than there were in the past.

Brachytherapy

Brachytherapy is the insertion of radiation seeds through thin tubes called catheters directly into the affected area of the body. Brachytherapy usually requires specialized skills and special training. It is only used in certain hospitals and only in special situations to treat sarcoma.

Proton beam radiation therapy

Proton therapy is a type of external-beam radiation treatment that uses protons rather than x-rays. Like x-rays, protons can destroy cancer cells. It is most commonly used in parts of the body close to critical structures, for example, near the spinal cord or at the base of the brain. Learn more about proton therapy. Radiation treatment using heavier charged particles, known as carbon-ion radiation therapy, is being studied for the treatment of sarcomas in Japan, Germany, and China.

Intraoperative radiation therapy

In some hospitals, part of the planned radiation therapy can be given during surgery. This approach can decrease the need to expose normal tissue to radiation from external-beam radiation or brachytherapy.

Side effects of radiation therapy

Side effects from radiation therapy depend on what part of the body receives radiation. They may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. In the short term, radiation can cause injury to the skin that looks like a sunburn. This is usually treated with creams that keep the skin soft and help relieve discomfort. Radiation therapy can also affect wound healing. In the long term, radiation can cause scarring that affects the function of an arm or a leg. In rare cases, radiation can cause another sarcoma or other cancer. In the unlikely event that this happens, it can be 7 to 20 years after radiation for a second cancer to develop. 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 side effects go away soon after treatment is finished.

Learn more about radiation therapy.

Therapies using medication

Systemic therapy is the use of medication to destroy cancer cells. This type of medication is given through the bloodstream to reach cancer cells throughout the body. Systemic therapies are generally prescribed by a medical oncologist, a doctor who specializes in treating cancer with medication.

Common ways to give systemic therapies include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally).

The types of systemic therapies used for sarcomas include:

  • Chemotherapy

  • Targeted therapy

  • Immunotherapy

Each of these types of therapies is discussed below in more detail. A person may receive 1 type of systemic therapy at a time or a combination of systemic therapies given at the same time. They can also be given as part of a treatment plan that includes surgery and/or radiation therapy.

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. It is also important to let your doctor know if you are taking any other prescription or over-the-counter medications or supplements. Herbs, supplements, and other drugs can interact with cancer medications. Learn more about your prescriptions by using searchable drug databases.

Chemotherapy

Chemotherapy is medication that keeps cancer cells from growing, dividing, and making more cells. Cancer cells grow and divide faster than normal cells. However, normal cells also grow and divide, so the side effects of chemotherapy are due to the treatment’s effects on normal cells that are growing and dividing.

A chemotherapy regimen, or schedule, usually consists of a specific number of cycles given over a set period of time. A patient may receive 1 drug at a time or a combination of different drugs given at the same time. Chemotherapy for sarcoma can usually be given as an outpatient treatment.

Different drugs are used to treat different types and subtypes of sarcoma. Some types of chemotherapy that might be used alone or in combination for STS include:

  • Doxorubicin (available as a generic drug)

  • Epirubicin (Ellence)

  • Ifosfamide (Ifex)

  • Gemcitabine (Gemzar)

  • Docetaxel (Taxotere)

  • Trabectedin (Yondelis)

  • Eribulin (Halaven)

  • Dacarbazine (available as a generic drug)

  • Liposomal doxorubicin (Doxil)

  • Methotrexate (Trexall)

  • Temozolomide (Temodar)

  • Vinblastine (Velban)

  • Vincristine (Vincasar)

In some cases, a specific drug or drugs are used for a particular type of sarcoma. Since there are over 50 types of STS, it is not possible to list them all here. Here are 2 examples:

For rhabdomyosarcoma and Ewing sarcoma of soft tissue or bone:

  • Doxorubicin

  • Ifosfamide

  • Cyclophosphamide (available as a generic drug)

  • Dactinomycin (Cosmegen)

  • Etoposide (available as a generic drug)

  • Irinotecan (Camptosar)

  • Temozolomide

  • Vincristine

For angiosarcoma:

  • Doxorubicin

  • Docetaxel

  • Paclitaxel (Taxol)

Chemotherapy is often used when a sarcoma has already spread. It may be given alone or in combination with surgery, radiation therapy, or both.

For example, certain types of sarcomas may be treated with chemotherapy before surgery to make the tumor easier to remove. 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.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These side effects usually go away after treatment is finished. In rare cases, there are long-term problems that affect the heart or kidneys or cause second cancers.

Learn more about the basics of chemotherapy.

Targeted therapy

Targeted therapy is a treatment that targets the cancer’s specific genes, 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 healthy cells.

Not all tumors have the same targets. To find the most effective treatment, your doctor may run tests to identify the genes, proteins, and other factors in your tumor. This helps doctors better match each patient with the most effective treatment whenever possible. In addition, research studies continue to find out more about specific molecular targets and new treatments directed at them. Learn more about the basics of targeted treatments.

In 2016, the U.S. Food and Drug Administration (FDA) gave accelerated approval for the use of olaratumab (Lartruvo) in combination with doxorubicin as the first treatment for people with STS that cannot be cured with radiation therapy or surgery. Unfortunately, in January 2019, the results of a larger clinical trial did not show any benefit to adding olaratumab to doxorubicin. Currently, it is not recommended that any people newly diagnosed with a sarcoma start olaratumab, unless it is in a clinical trial.

Imatinib (Gleevec) is a type of targeted therapy called a tyrosine kinase inhibitor. It is the standard first-line treatment for GIST worldwide. Imatinib is approved for use for patients with GIST after initial surgery to try to prevent recurrence in those who might have a high risk of recurrence. Imatinib is also approved for the treatment of people with advanced-stage dermatofibrosarcoma protuberans (DFSP). Two other targeted drugs, sunitinib (Sutent) and regorafenib (Stivarga), are approved for the treatment of GIST when imatinib doesn’t work.

Pazopanib (Votrient) is a type of targeted therapy called a multitargeted tyrosine kinase inhibitor. It received FDA approval to treat kidney cancer in 2006 and STS in 2012.

Pexidartinib (Turalio) is a colony-stimulating factor-1 (CSF-1) inhibitor that the FDA has approved to treat certain people with tenosynovial giant cell tumors (TGCT), sometimes called pigmented villonodular synovitis or giant cell tumor of tendon sheath. These are rare tumors that affect the tendons and joints of younger adults. In a recent clinical trial, of the 61 people who took pexidartinib, the drug worked in 39.3% of them and improved their pain, range of motion, and physical function. Pexidartinib can be used to treat people with significant symptoms from TGCT for whom surgery is not considered a good option. Taking pexidartinib did cause serious or potentially fatal side effects that affected the liver in some patients. Therefore, it can only be prescribed by a certified specialist, and patients must enroll in a patient registry.

A small percentage of sarcomas, less than 1%, have a mutation in the neurotrophic receptor tyrosine kinase (NTRK) gene. Larotrectinib (Vitrakvi) is an NTRK inhibitor that is now approved for any cancer that has a specific mutation in the NTRK gene. The most common side effects include fatigue, nausea, dizziness, vomiting, increased liver enzymes, cough, constipation, and diarrhea.

Immunotherapy

Immunotherapy, also called biologic therapy, is designed to boost the body's natural defenses to fight the cancer. It uses materials made either by the body or in a laboratory to improve, target, or restore immune system function.

Immunotherapy is generally not approved for the treatment of sarcomas because they have not been tested well enough. Many recently approved immunotherapy treatments for other types of cancers involve “immune checkpoint inhibitors.” These drugs are given to take the brakes off the body’s natural immune response against the cancer in the body. The current methods of immunotherapy do have problems because these drugs also activate immune responses against normal body parts, a process called autoimmunity. Some of these drugs are approved to treat other cancers. However, if testing on your tumor shows that it has specific problems with repairing DNA damage, called microsatellite instability-high (MSI-H) or mismatch repair deficiency (dMMR; this occurs in less than 1% of sarcomas), then a checkpoint inhibitor called pembrolizumab (Keytruda) may be used. In general, checkpoint inhibitors and other immunotherapies to treat sarcoma are still being studied and only considered in clinical trials.

Different types of immunotherapy can cause different side effects. Common side effects include skin reactions, flu-like symptoms, diarrhea, and weight changes. Talk with your doctor about possible side effects for the immunotherapy recommended for you. Learn more about the basics of immunotherapy.

Physical, emotional, and social effects of cancer

Cancer and its treatment cause physical symptoms and side effects, as well as emotional, social, and financial effects. Managing all of these effects is called palliative care or supportive care. It is an important part of your care that is included along with treatments intended to slow, stop, or eliminate the cancer.

Palliative care focuses on improving how you feel during treatment by managing symptoms and supporting patients and their families with other, non-medical needs. Any person, regardless of age or type and stage of cancer, may receive this type of care. And it often works best when it is started right after a cancer diagnosis. People who receive palliative care along with treatment for the cancer often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, emotional and spiritual support, and other therapies. You may also receive palliative treatments similar to those meant to get rid of the cancer, such as chemotherapy, surgery, or radiation therapy.

Before treatment begins, talk with your doctor about the goals of each treatment in the treatment plan. You should also talk about the possible side effects of the specific treatment plan and palliative care options.

During treatment, your health care team may ask you to answer questions about your symptoms and side effects and to describe each problem. Be sure to tell the health care team if you are experiencing a problem. This helps the health care team treat any symptoms and side effects as quickly as possible. It can also help prevent more serious problems in the future.

Learn more about the importance of tracking side effects in another part of this guide. Learn more about palliative care in a separate section of this website.

Treatment options for sarcoma by stage

When considering the treatment plan, doctors will often divide sarcomas into 2 categories: curable and treatable. Curable sarcomas can be completely removed from the body, with the goal of preventing it from coming back. Treatable sarcomas cannot be totally removed from the body but can be controlled with treatments. In many cases, stage I to stage III sarcoma is curable and stage IV, or metastatic, sarcoma is treatable.

Stage I sarcoma

At this early stage, sarcoma can often be completely removed with surgery. Treatment with radiation therapy before and/or after surgery may be recommended.

Stage II sarcoma

Stage II sarcoma is often high grade and can grow and spread quickly. Treatments at this stage include surgery plus radiation therapy. If the tumor is hard to reach, radiation therapy may be used first to shrink the tumor. This is called neoadjuvant treatment. Or, if the tumor can be removed with surgery, radiation therapy may be used afterward to reduce the risk of the cancer coming back. This is called adjuvant treatment.

Stage III sarcoma

Stage III sarcoma is also high grade and larger. Treatment will involve a combination of surgery and radiation therapy. Chemotherapy may also be added to the treatment plan. Radiation therapy, chemotherapy, or both may be given before and/or after surgery to shrink tumors and lower the risk of the cancer coming back.

Metastatic (stage IV) sarcoma

If cancer spreads to another part in the body from where it started, doctors call it metastatic cancer. If this happens, it is a good idea to talk with doctors who have experience in treating it. Doctors can have different opinions about the best standard treatment plan. Clinical trials might also be an option. Learn more about getting a second opinion at a sarcoma specialty center before starting treatment, so you are comfortable with your chosen treatment plan.

Your treatment plan may include medications as described above and other palliative treatments to help relieve symptoms and side effects. In addition to providing symptom relief, medical treatments such as chemotherapy may also slow the spread of the cancer. The type of medical treatment that is recommended will depend on many factors, such as the type of sarcoma, which treatments you have received before, and your medical history. Clinical trials involving new drugs or combinations of drugs may also be considered.

Surgery may be used to remove individual metastases, especially if the cancer has spread to a lung, but only a small percentage of people benefit from this. This surgical procedure is called metastatectomy. Radiation therapy can also be used to help relieve symptoms and side effects. If cancer has spread to the liver, localized treatments may be recommended, such as surgery, radiation therapy, and chemotherapy. Occasionally, when the tumor is not growing, a “watch and wait” approach, also called “active surveillance,” may be used. This means that the patient is closely monitored and active treatment begins only if the tumor begins to grow.

For most people, a diagnosis of metastatic cancer is very stressful and, at times, difficult to bear. You and your family are encouraged to talk about how you feel with doctors, nurses, social workers, or other members of the health care team. It may also be helpful to talk with other patients, possibly through a support group.

Remission and the chance of recurrence

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

A remission may be temporary or permanent. This uncertainty causes many people to worry that the cancer will come back. While many remissions are permanent, it is important to talk with your doctor about the possibility of the cancer returning. Understanding your 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 returns after the original treatment, it is called recurrent cancer. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence). If the sarcoma was originally in the arm or leg, the recurrence most commonly occurs in the lungs. Patients treated for sarcoma of the abdomen or torso are at risk for local, regional, or distant recurrence.

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

A local recurrence often can be successfully treated with additional surgery plus radiation therapy, but the risks of side effects from these treatments tends to increase. Treatment for a distant recurrence is most successful when there are a small number of tumors that have spread to the lung that can be completely removed surgically, destroyed with radiofrequency ablation (see below), or destroyed with ablative high-dose radiation therapy (also known as stereotactic body radiotherapy, SBRT, or gamma-knife radiotherapy):

  • Radiofrequency ablation is a technique where a needle is inserted into the tumor to deliver an electrical current that can destroy the cancer. This burns the tumor from the inside out.

  • SBRT is the use of pinpointed radiation at very high doses over a few treatments to attack a specific small area of tumor. This is a useful technique because it uses fewer treatments and can be more precise than external-beam radiation therapy.

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

If treatment does not work

Recovery from cancer is not always possible. If the cancer cannot be cured, it can often still be controlled, at least for a period of time. It is important to understand that patients can live with cancer in their body as long as it does not affect the function of a major organ. Therefore, the goal of treatment is to control the cancer and preserve organ function.

If the cancer can no longer be controlled, it is called end-stage or terminal cancer. This diagnosis is stressful, and advanced cancer is difficult to discuss for many people. However, it is important to have open and honest conversations with your health care team to express your feelings, preferences, and concerns. The health care team has special skills, experience, and knowledge to support patients and their families and is there to help. Making sure a person is physically comfortable, free from pain, and emotionally supported is extremely important.

People who have advanced cancer and who are expected to live fewer than 6 months may want to consider hospice care. Hospice care is designed to provide the best possible quality of life for people who are near the end of life. You and your family are encouraged to talk with the health care team about hospice care options, which include hospice care at home, a special hospice center, or other health care locations. Nursing care and special equipment can make staying at home a workable option 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 loss.

The next section in this guide is About Clinical Trials. It offers more information about research studies that are focused on finding better ways to care for people with cancer. Use the menu to choose a different section to read in this guide.

Sarcoma, Soft Tissue - About Clinical Trials

Approved by the Cancer.Net Editorial Board, 05/2019

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. Use the menu to see other pages.

What are clinical trials?

Doctors and scientists are always looking for better ways to care for people with sarcoma. To make scientific advances, doctors create research studies involving volunteers, called clinical trials. In fact, every drug that is now approved by the U.S. Food and Drug Administration (FDA) was tested in clinical trials.

Clinical trials are used for all types and stages of sarcoma. Many focus on new treatments to learn if a new treatment is safe, effective, and possibly better than the existing treatments. These types of studies evaluate new drugs, different combinations of treatments, new approaches to radiation therapy or surgery, and new methods of treatment.

People who participate in clinical trials can be some of the first to get a treatment before it is available to the public. However, there are some risks with a clinical trial, including possible side effects and the chance that the new treatment may not work. People are encouraged to talk with their health care team about the pros and cons of joining a specific study.

Some clinical trials study new ways to relieve symptoms and side effects during treatment. Others study ways to manage the late effects that may happen a long time after treatment. Talk with your doctor about clinical trials for symptoms and side effects.

Deciding to join a clinical trial

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

Insurance coverage and the costs of clinical trials differ by location and by study. In some programs, some of the expenses from participating in the clinical trial are reimbursed. In others, they are not. It is important to talk with the research team and your insurance company first to learn if and how your treatment in a clinical trial will be covered. Learn more about health insurance coverage of clinical trials.

Sometimes people have concerns that, in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” When used, placebos are usually combined with standard treatment in most cancer clinical trials. 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.

Patient safety and informed consent

To join a clinical trial, people must participate in a process known as informed consent. During informed consent, the doctor should:

  • Describe all of the treatment options so that the person understands how the new treatment differs from the standard treatment.

  • List all of the risks of the new treatment, which may or may not be different from the risks of standard treatment.

  • Explain what will be required of each person in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

Clinical trials also have certain rules called “eligibility criteria” that help structure the research and keep patients safe. You and the research team will carefully review these criteria together.

People who participate in a clinical trial may stop participating at any time for personal or medical reasons. 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 people 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 they choose to leave the clinical trial before it ends.

Finding a clinical trial

Research through clinical trials is ongoing for all types of cancer. For specific topics being studied for sarcoma, learn more in the Latest Research section.

Cancer.Net offers a lot of information about cancer clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of cancer.

PRE-ACT, Preparatory Education About Clinical Trials

In addition, you can find a free video-based educational program about cancer clinical trials in another section of this website.

The next section in this guide is Latest Research. It explains areas of scientific research for sarcoma. Use the menu to choose a different section to read in this guide.

Sarcoma, Soft Tissue - Latest Research

Approved by the Cancer.Net Editorial Board, 05/2019

ON THIS PAGE: You will read about the scientific research being done to learn more about sarcomas and how to treat it. Use the menu to see other pages.

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 best diagnostic and treatment options for you.

  • Immunotherapy. Immunotherapy comes in many forms. Immune checkpoint inhibitors have become the focus of a lot of research. These drugs are targeted monoclonal antibodies (proteins) that turn on immune responses in the body by taking the brakes off the immune system. The molecules that are blocked have names such as CTLA4, PD-1, PD-L1, OX40, LAG3, and TIM3. This approach has proved helpful in treating many cancers and in research studies about sarcomas.

    Chimeric antigen receptor (CAR) T-cell therapy uses white blood cells from a patient’s blood to destroy cancer cells. The patient’s cells are removed the patient and then changed in a laboratory so they have specific proteins called receptors. These receptors allow those T cells to recognize the cancer cells. The changed T cells are grown in large numbers in the laboratory and returned to the patient’s body. Once in the patient’s body, these T cells seek out and destroy cancer cells. This technique shows the most promise when there is a known target on the sarcoma, such as in synovial sarcoma and myxoid-round cell liposarcoma. Clinical trials are investigating this exciting but complex form of immunotherapy.

    Vaccines against specific sarcoma proteins or other molecules are also being studied, often in addition to immune checkpoint inhibitors.

  • Improved drug delivery. Some chemotherapies are incorporated into fat molecules called liposomes to improve the absorption and distribution of the drug in the patient’s body. Other new ways to get a chemotherapy into a cancer cell are being studied. For example, chemotherapy can be attached to proteins, so the chemotherapy can enter into the cancer cells. In some cases, these proteins are antibodies that target, or bind to, a specific marker on a cancer cell, so the chemotherapy is delivered only to cancer cells and not to normal cells, which can improve effectiveness and decrease side effects.

  • New drugs. New medications are being developed and tested that may be effective in treating some subtypes of STS. Learn more about the process of drug development and approval.

  • Targeted therapy. As explained in Types of Treatment, several targeted therapies have been recently approved to treat specific types of sarcoma. This is an active area of research for sarcoma.

    A recent study examined using sorafenib (Nexavar) to treat a rare type of sarcoma called a desmoid tumor. Sorefenib is a tyrosine kinase inhibitor currently used to treat liver, thyroid, and kidney cancers. Of those who took sorafenib in the clinical trial, 87% had their disease stopped for more than 1 year. In this study, 18% of patients who took sorafenib had to stop taking the drug because of side effects.

  • Targeted oncogene treatments. Drugs are being researched that may block 1 or more of the proteins found in tumor cells that help the tumor grow and spread.

  • Tumor genetics. Researchers are learning that some sarcomas have unique genetic “fingerprints.” Understanding these fingerprints may help doctors make precise diagnoses, determine better treatments, and possibly better predict a patient’s prognosis. A number of cancer centers and companies now offer genetic tests of cancers to determine whether people with a sarcoma might benefit from newer treatments.

  • Palliative care/supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current sarcoma treatments to improve comfort and quality of life for patients.

Looking for More About the Latest Research?

If you would like additional information about the latest areas of research regarding sarcoma, explore these related items that take you outside of this guide:

The next section in this guide is Coping with Treatment. It offers some guidance in how to cope with the physical, emotional, social, and financial changes that cancer and its treatment can bring. Use the menu to choose a different section to read in this guide.

Sarcoma, Soft Tissue - Coping with Treatment

Approved by the Cancer.Net Editorial Board, 05/2019

ON THIS PAGE: You will learn more about coping with the physical, emotional, social, and financial effects of cancer and its treatment. Use the menu to see other pages.

Every cancer treatment can cause side effects or changes to your body and how you feel. For many reasons, people do not experience the same side effects even when they are given the same treatment for the same type of cancer. This can make it hard to predict how you will feel during treatment.

As you prepare to start cancer treatment, it is normal for some people to fear treatment-related side effects. It may help to know that your health care team will work to prevent and relieve side effects. Doctors call this part of cancer treatment “palliative care" or "supportive care.” It is an important part of your treatment plan, regardless of your age or the stage of disease.

Coping with physical side effects

Common physical side effects from each treatment option for sarcomas are listed in the Types of Treatment section. Learn more about side effects of cancer and its treatment, along with ways to prevent or control them. Changes to your physical health depend on several factors, including the cancer’s stage, the length and dose of treatment, and your general health.

Talk with your health care team regularly about how you are feeling. It is important to let them know about any new side effects or changes in existing side effects. If they know how you are feeling, they can find ways to relieve or manage your side effects to help you feel more comfortable and potentially keep any side effects from worsening.

You may find it helpful to keep track of your side effects so it is easier to explain any changes with your health care team. Learn more about why tracking side effects is helpful.

Sometimes, physical side effects can last after treatment ends. Doctors call these long-term side effects. They call side effects that occur months or years after treatment late effects. Treating long-term side effects and late effects is an important part of survivorship care. Learn more by reading the Follow-up Care section of this guide or talking with your doctor.

Coping with emotional and social effects

You can have emotional and social effects as well as physical effects after a cancer diagnosis. This may include dealing with difficult emotions, such as sadness, anxiety, or anger, or managing your stress level. Sometimes, people find it difficult to express how they feel to their loved ones. Some have found that talking to an oncology social worker, counselor, or member of the clergy can help them develop more effective ways of coping and talking about cancer.

You can also find coping strategies for emotional and social effects in a separate section of this website. This section includes many resources for finding support and information to meet your needs.

Coping with financial effects

Cancer treatment can be expensive. It is often a big source of stress and anxiety for people with cancer and their families. In addition to treatment costs, many people find they have extra, unplanned expenses related to their care. For some people, the high cost of medical care stops them from following or completing their cancer treatment plan. This can put their health at risk and may lead to higher costs in the future. Patients and their families are encouraged to talk about financial concerns with a member of their health care team. Learn more about managing financial considerations in a separate part of this website.

Caring for a loved one with cancer

Family members and friends often play an important role in taking care of a person with sarcoma. This is called being a caregiver. Caregivers can provide physical, practical, and emotional support to the patient, even if they live far away.

Caregivers may have a range of responsibilities on a daily or as-needed basis, including:

  • Providing support and encouragement

  • Talking with the health care team

  • Giving medications

  • Helping manage symptoms and side effects

  • Coordinating medical appointments

  • Providing a ride to and from appointments

  • Assisting with meals

  • Helping with household chores

  • Handling insurance and billing issues

Learn more about caregiving.

Talking with your health care team about side effects

Before starting treatment, talk with your doctor about possible side effects. Ask:

  • Which side effects are most likely?

  • When are they likely to happen?

  • What can we do to prevent or relieve them?

Be sure to tell your health care team about any side effects that happen during treatment and afterward, too. Tell them even if you do not think the side effects are serious. This discussion should include physical, emotional, and social effects of cancer.

Also, ask how much care you may need at home and with daily tasks during and after treatment. This can help you make a caregiving plan. Create a caregiving plan with this 1-page fact sheet that includes an action plan to help make caregiving a team effort. This free fact sheet is available as a PDF, so it is easy to print out.

Looking for More on How to Track Side Effects?

Cancer.Net offers several resources to help you keep track of your symptoms and side effects. Please note that these links will take you to other sections of Cancer.Net:

  • Cancer.Net Mobile: The free Cancer.Net mobile app allows you to securely record the time and severity of symptoms and side effects.

  • ASCO Answers Managing Pain: Get this 36-page booklet about the importance of pain relief that includes a pain tracking sheet to help patients record how pain affects them. The free booklet is available as a PDF, so it is easy to print out.

  • ASCO Answers Fact Sheets: Read 1-page fact sheets on diarrhea and rash that provide a tracking sheet to record the timing and severity of the side effect. These free fact sheets are available as a PDF, so they are easy to print out.

The next section in this guide is Follow-up Care. It explains the importance of checkups after cancer treatment is finished. Use the menu to choose a different section to read in this guide.

Sarcoma, Soft Tissue - Follow-Up Care

Approved by the Cancer.Net Editorial Board, 05/2019

ON THIS PAGE: You will read about your medical care after cancer treatment is completed and why this follow-up care is important. Use the menu to see other pages.

Care for people diagnosed with cancer does not end when active treatment has finished. Your health care team will continue to check that the cancer has not come back, manage any side effects, and monitor your overall health. This is called follow-up care.

Your follow-up care may include regular physical examinations, medical tests, or both. Doctors want to keep track of your recovery in the months and years ahead. For patients treated for a 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 lymphedema may be recommended. A rehabilitation medicine specialist can help patients receive the most appropriate rehabilitation after treatment. The majority of patients with a sarcoma in an arm or leg can be successfully treated and maintain good limb function. However, when treatment included amputation, services that provide artificial limbs, called prosthetics, and additional mental health support can help manage the adjustment to life following the loss of a limb. Learn more about rehabilitation.

Learn more about the importance of follow-up care.

Watching for recurrence

One goal of follow-up care is to check for a recurrence, which means that the cancer has come back. Cancer recurs because small areas of cancer cells may remain undetected in the body. Over time, these cells may increase in number until they show up on test results or cause signs or symptoms. During follow-up care, a doctor familiar with your medical history can give you personalized information about your risk of recurrence. Your doctor will ask specific questions about your health. Some people may have blood tests or imaging tests done as part of regular follow-up care, but testing recommendations depend on several factors, including the type and stage of cancer originally diagnosed and the types of treatment given.

The anticipation before having a follow-up test or waiting for test results can add stress to you or a family member. This is sometimes called “scan-xiety.” Learn more about how to cope with this type of stress.

Managing long-term and late side effects

Most people expect to experience side effects when receiving treatment. However, it is often surprising to survivors that some side effects may linger beyond the treatment period. These are called long-term side effects. Other side effects called late effects may develop months or even years afterwards. Long-term and late effects can include both physical and emotional changes.

Talk with your doctor about your risk of developing such side effects based on the type of cancer, your individual treatment plan, and your overall health. If you had a treatment known to cause specific late effects, you may also have certain physical examinations, scans, or blood tests to help find and manage them.

Keeping personal health records

You and your doctor should work together to develop a personalized follow-up care plan. Be sure to discuss any concerns you have about your future physical or emotional health. ASCO offers forms to help keep track of the cancer treatment you received and develop a survivorship care plan when treatment is completed.

This is also a good time to talk with your doctor about who will lead your follow-up care. Some survivors continue to see their oncologist, while others transition back to the care of their family doctor or another health care professional. This decision depends on several factors, including the type and stage of cancer, side effects, health insurance rules, and your personal preferences.

If a doctor who was not directly involved in your cancer care will lead your follow-up care, be sure to share your cancer treatment summary and survivorship care plan forms with him or her and with all future health care providers. Details about your cancer treatment are very valuable to the health care professionals who will care for you throughout your lifetime.

The next section in this guide is Survivorship. It describes how to cope with challenges in everyday life after a cancer diagnosis. Use the menu to choose a different section to read in this guide.

Sarcoma, Soft Tissue - Survivorship

Approved by the Cancer.Net Editorial Board, 05/2019

ON THIS PAGE: You will read about how to cope with challenges in everyday life after a cancer diagnosis. Use the menu to see other pages.

What is survivorship?

The word “survivorship” means different things to different people. Common definitions include:

  • Having no signs of cancer after finishing treatment.

  • Living with, through, and beyond cancer. According to this definition, cancer survivorship begins at diagnosis and includes people who continue to have treatment over the long term, to either reduce the risk of recurrence or to manage chronic disease.

Survivorship is one of the most complicated parts of having cancer. This is because it is different for everyone.

Survivors may experience a mixture of strong feelings, including joy, concern, relief, guilt, and fear. Some people say they appreciate life more after a cancer diagnosis and have gained a greater acceptance of themselves. Others become very anxious about their health and uncertain about coping with everyday life.

Survivors may feel some stress when their frequent visits to the health care team end after completing treatment. Often, relationships built with the cancer care team provide a sense of security during treatment, and people miss this source of support. This may be especially true when new worries and challenges surface over time, such as any late effects of treatment, emotional challenges including fear of recurrence, sexual health and fertility concerns, and financial and workplace issues.

Every survivor has individual concerns and challenges. With any challenge, a good first step is being able to recognize your fears and talk about them. Effective coping requires:

  • Understanding the challenge you are facing

  • Thinking through solutions

  • Asking for and allowing the support of others

  • Feeling comfortable with the course of action you choose

Many survivors find it helpful to join an in-person support group or an online community of survivors. This allows you to talk with people who have had similar first-hand experiences. Other options for finding support include talking with a friend or member of your health care team, individual counseling, or asking for assistance at the learning resource center of the place where you received treatment.

People with a family history of cancer

Some people with sarcoma have strong family histories of cancer in other relatives. If other relatives in your family have had cancer, then it may be a good idea to meet with a genetic counselor. Genetic counselors can discuss what your diagnosis may mean for other people in your family who do not have cancer. Sometimes, special blood-test screening can determine who is at risk to develop cancer in a family. Learn more about genetic testing for cancer risk.

Changing role of caregivers

Family members and friends may also go through periods of transition. A caregiver plays a very important role in supporting a person diagnosed with cancer, providing physical, emotional, and practical care on a daily or as-needed basis. Many caregivers become focused on providing this support, especially if the treatment period lasts for many months or longer.

However, as treatment is completed, the caregiver's role often changes. Eventually, the need for caregiving related to the cancer diagnosis will become much less or come to an end. Caregivers can learn more about adjusting to life after caregiving.

A new perspective on your health

For many people, survivorship serves as a strong motivator to make lifestyle changes.

People recovering from sarcoma are encouraged to follow established guidelines for good health, such as not smoking, limiting alcohol, eating well, and managing stress. Regular physical activity can help rebuild your strength and energy level. Your health care team can help you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about making healthy lifestyle choices.

It is important to have recommended medical checkups and tests (see Follow-up Care) to take care of your health. Cancer rehabilitation may be recommended, and this could mean any of a wide range of services such as physical therapy, career counseling, pain management, nutritional planning, and/or emotional counseling. The goal of rehabilitation is to help people regain control over many aspects of their lives and remain as independent and productive as possible.

Talk with your health care team to develop a survivorship care plan that is best for your needs.

Looking for More Survivorship Resources?

For more information about cancer survivorship, explore these related items. Please note that these links will take you to other sections of Cancer.Net:

  • ASCO Answers Cancer Survivorship Guide: Get this 44-page booklet that helps people transition into life after treatment. It includes blank treatment summary and survivorship care plan forms. The free booklet is available as a PDF, so it is easy to print out.

  • Cancer.Net Patient Education Video: View a short video led by an ASCO expert that provides information about what comes after finishing treatment.

  • Survivorship Resources: Cancer.Net offers an entire area of this website with resources to help survivors, including for those in different age groups.

The next section offers Questions to Ask the Health Care Team to help start conversations with your cancer care team. Use the menu to choose a different section to read in this guide.

Sarcoma, Soft Tissue - Questions to Ask the Health Care Team

Approved by the Cancer.Net Editorial Board, 05/2019

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

Talking often with the health care team 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. You may want to print this list and bring it to your next appointment, or download Cancer.Net’s free mobile app for a digital list and other interactive tools to manage your care. It may also be helpful to ask a family member or friend to come with you to appointments to help take notes.

Questions to ask after getting a diagnosis

  • What type of sarcoma do I have?

  • Has this diagnosis been reviewed by a pathologist who is experienced in the diagnosis and classification of sarcoma?

  • Can you explain my pathology report (laboratory test results) to me?

  • What stage and grade is the sarcoma? What does this mean?

  • What does my diagnosis mean?

  • Where is the tumor located?

  • Should I see a sarcoma specialist?

  • Was my case discussed in a tumor board or other multidisciplinary conference? What does this mean?

Questions to ask about choosing a treatment and managing side effects

  • What are my treatment options?

  • What clinical trials are available for me? Where are they located, and how do I find out more about them?

  • What treatment plan do you recommend? Why?

  • What is the goal of each treatment? Is it to eliminate the cancer, help me feel better, or both?

  • What are the possible side effects of this treatment, both in the short term and the long term?

  • Who will be part of my health care team, and what does each member do?

  • Who will be leading my overall treatment?

  • 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 of cancer care, who can help me?

  • What support services are available to me? To my family?

  • If I have questions or problems, who should I call?

Questions to ask about having surgery

  • Should I have a biopsy before surgery?

  • Does the stage of cancer affect the decision for surgery? How?

  • What will happen during this surgery?

  • How long will the operation take?

  • How long will I be in the hospital?

  • Will the surgery be performed in en-bloc resection? What does this mean?

  • How much experience does this surgeon have 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?

  • Who should I contact about any side effects I experience? And how soon?

  • Do I need chemotherapy and/or radiation therapy before the surgery? After the surgery?

  • Will I need rehabilitation after the surgery?

  • If surgery is on an arm or leg, what will it look like after surgery?

Questions to ask about having 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?

  • Who should I contact about any side effects I experience? And how soon?

  • What can be done to prevent or to treat the side effects?

Questions to ask about having systemic therapy using medication

  • What type of treatment is recommended?

  • What is the goal of this treatment?

  • How experienced is the medical oncologist with sarcoma? How many people with sarcoma has he or she treated in the past year?

  • Are other chemotherapy or targeted treatments available?

  • Should I consider a clinical trial?

  • How many treatments will I need? How long will each take?

  • What side effects can I expect during treatment?

  • Who should I contact about any side effects I experience? And how soon?

  • What are the possible long-term effects of having this treatment?

  • What can be done to relieve side effects?

Questions to ask about planning follow-up care

  • What is the chance that the cancer will come back? Should I watch for specific signs or symptoms?

  • What long-term side effects or late effects are possible based on the cancer treatment I received?

  • What follow-up tests will I need, and how often will I need them?

  • How do I get a treatment summary and survivorship care plan to keep in my personal records?

  • Who will be leading my follow-up care?

  • What survivorship support services are available to me? To my family?

The next section in this guide is Additional Resources. It offers some more resources on this website that may be helpful to you. Use the menu to choose a different section to read in this guide.

Sarcoma, Soft Tissue - Additional Resources

Approved by the Cancer.Net Editorial Board, 05/2019

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 Soft-Tissue Sarcoma. Use the menu to go back and see other pages.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer for the person diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond.

Here are a few links to help you explore other parts of Cancer.Net:

This is the end of Cancer.Net’s Guide to Soft-Tissue Sarcoma. Use the menu to choose a different section to read in this guide.