Sarcoma is a type of cancer that affects the body’s soft tissue and bones. This section focuses on soft tissue sarcoma. 

Sarcoma is a rare form of cancer in adults, representing about 1% of adult cancers. It’s more common in children and accounts for 15% of childhood cancers.

The body’s soft tissue includes: 

  • Muscles
  • Fat
  • Nerves
  • Tissue around the joints (synovial tissue)
  • Tendons (bands of tissue that connect muscles to bones)
  • Fibrous tissue
  • Lymph vessels
  • Blood vessels
  • Mix of cartilage and bone

Sarcoma can occur nearly anywhere on the body. Approximately:

  • 60% of cases occur in the leg or arm
  • 30% of cases begin in the abdomen or torso
  • 10% of cases occur in the head or neck 

Benign (noncancerous) tumors can also start in the soft tissues, though these are not sarcomas.

Types of Soft Tissue Sarcoma

Soft tissue sarcoma is a family of more than 50 different diseases. Each type of sarcoma is named for the normal tissue cells the cancer cells most closely resemble. The most common types of soft tissue sarcoma include:

  • Adult fibrosarcoma
  • Alveolar soft part sarcoma
  • Angiosarcoma
  • Clear cell sarcoma
  • Desmoplastic small round cell tumor
  • Epithelioid sarcoma
  • Fibromyxoid sarcoma
  • Gastrointestinal stromal tumor (GIST)
  • Kaposi sarcoma
  • Leiomyosarcoma
  • Liposarcomas
  • Malignant mesenchymoma
  • Malignant peripheral nerve sheath tumors include neurofibrosarcomas, malignant schwannomas, and neurogenic sarcomas
  • Myxofibrosarcoma
  • Rhabdomyosarcoma
  • Synovial sarcoma
  • Undifferentiated pleomorphic sarcoma (UPS)

Adult Fibrosarcoma

Adult fibrosarcoma can develop in the arms, legs or trunk (abdomen, back, chest and pelvis). It can affect people of any age, including infants, though it’s most common between the ages of 20 and 60.

Alveolar Soft Part Sarcoma

Alveolar soft part sarcoma is a rare disease that most often occurs in the legs. It’s most common in young adults. 


Angiosarcoma begins in the lymph vessels (lymphangiosarcomas) or the blood vessels (hemangiosarcomas). Angiosarcoma tumors sometimes form in parts of the body that have been treated with radiation therapy. These tumors can also develop in areas affected by lymphedema, such as the arms or legs.

Clear Cell Sarcoma

Clear cell sarcoma is a rare form of cancer that begins in the tendons of the arms or legs. When viewed under a microscope, clear cell sarcoma cells have some features of malignant melanoma cells (a form of skin cancer). It’s unclear why these cancers start in parts of the body other than the skin.

Desmoplastic Small Round Cell Tumor

Desmoplastic small round cell tumor is a rare form of sarcoma in young adults and teenagers. It most often develops in the abdomen (belly).

Epithelioid Sarcoma

Epithelioid sarcoma is most common in teens and young adults. It affects the tissue under the skin in the feet, hands, lower legs and forearms.

Fibromyxoid Sarcoma

Fibromyxoid sarcoma (low-grade) is a slow-growing disease that starts as a painless lump in the trunk, arms or legs (particularly in the thigh). Also called an Evans’ tumor, this type of sarcoma is more common in young and middle-aged adults.

Gastrointestinal Stromal Tumor (GIST)

Gastrointestinal stromal tumor is a form of sarcoma that begins in the digestive tract.

Kaposi Sarcoma

Kaposi sarcoma is a type of sarcoma that begins in the cells that line the blood vessels or lymph vessels.


Leiomyosarcoma is a disease that begins in the smooth muscle tissue. It most often occurs in the abdomen (belly), but can also develop in the uterus, arms or legs.


Liposarcomas are cancerous tumors in fat tissue. They can occur anywhere in the body, though they most often start behind the knee, inside the back of the belly or in the thigh. Liposarcomas are more common in adults between the ages of 50 and 65.

Malignant Mesenchymoma

Malignant mesenchymoma is a rare disease that has the characteristics of fibrosarcoma and at least two other kinds of sarcoma.

Malignant Peripheral Nerve Sheath Tumors

Malignant peripheral nerve sheath tumors begin in the cells that surround the nerves. Malignant peripheral nerve sheath tumors include:

  • Malignant schwannomas
  • Neurogenic sarcomas
  • Neurofibrosarcomas


Myxofibrosarcoma (low-grade) most often occurs in the arms and legs of older adults. These tumors typically develop in the skin or just under the skin. Sometimes, people develop more than one tumor.


Rhabdomyosarcoma is a form of childhood sarcoma that typically starts in the muscles that attach to the bones, though it can occur elsewhere in the body.

Synovial Sarcoma

Synovial sarcoma affects the tissue around the joints. It most commonly affects the shoulder, hip, knee and ankle joints. It’s more common in children and young adults, though it can occur in people of all ages.

Undifferentiated Pleomorphic Sarcoma (UPS)

Undifferentiated pleomorphic sarcoma (UPS) was previously called malignant fibrous histiocytoma. It typically occurs in the arms or legs, though it can also start in the back of the abdomen. UPS can grow into nearby and distant parts of the body. It is more common in older adults.

To understand sarcoma, it’s first important to understand what cancer is: basically, the production of abnormal cells. 

The body is programmed to routinely replenish cells in different organs. As normal cells age or get damaged, they die off. New cells take their place. This is what’s supposed to happen.

Abnormal cell growth refers to a buildup of extra cells. This happens when: 

  • New cells form even though the body doesn’t need them
  • Old, damaged cells don’t die off 

These extra cells slowly accumulate to form a tissue mass, lump or growth called a tumor. These abnormal cells can destroy normal body tissue and spread through the bloodstream and lymphatic system.

The first thing to understand is that a growth can be either benign or malignant. You’ve probably heard these terms. Here’s exactly what they mean:

  • Benign means not cancerous. A benign tumor can grow larger but does not spread to other tissues or organs. Benign tumors:
    • Can be removed
    • Usually don’t grow back
    • Are rarely fatal
    • Don’t spread to other tissues or body parts
  • Malignant means cancerous. The cells of a malignant tumor can spread and invade nearby tissues and organs. They are destructive. Malignant tumors:
    • Can often be removed
    • Sometimes grow back
    • Can invade and damage other tissues and organs
    • Can spread to other body parts
    • Can be fatal

Soft tissue sarcoma is relatively rare, accounting for only 1% of adult cancers and 15% of childhood cancers. The American Cancer Society (ACS) estimates that in 2022, approximately 13,000 new soft tissue sarcomas will be diagnosed in adults and children. By comparison, the ACS estimates that approximately 268,000 cases of prostate cancer and 287,000 cases of invasive breast cancer will be diagnosed in the same year.

The cause of most sarcomas is unknown, though some factors can increase your risk. Keep in mind that a risk factor doesn’t mean you’ll develop sarcoma.

Soft tissue sarcoma risk factors include:

  • Prior radiation therapy. Radiation therapy treatment for a previous cancer (usually five years ago or more) may slightly raise your risk of developing sarcoma in the area that was treated.
  • Certain inherited diseases can increase the risk of sarcoma, including familial adenomatous polyposis (also called Gardner syndrome), Li-Fraumeni syndrome, neurofibromatosis type 1 (also known as von Recklinghausen’s disease), nevoid basal cell carcinoma syndrome, retinoblastoma, tuberous sclerosis complex and Werner syndrome.
  • Compromised immune system. Conditions that affect the immune system—such as human immunodeficiency virus (HIV), lupus, psoriasis and chronic lymphocytic leukemia—can increase the risk of soft tissue sarcoma.
  • Exposure to certain chemicals. While most sarcomas aren’t associated with environmental hazards, exposure to dioxin, Agent Orange or vinyl chloride monomer (used to make some plastics) may increase your risk.
  • When the lymph nodes are damaged or removed by surgery or radiation therapy, lymph fluid can build up in the body and cause swelling. This condition is known as lymphedema. Lymphedema typically occurs in the arms or legs, though it can develop elsewhere in the body. Sometimes, sarcomas form in parts of the body with lymphedema.

Lifestyle factors like exercise, diet and smoking aren’t linked to an increased or decreased risk of sarcoma.

Talk to your physician if you have any of these risk factors.

Because the cause of most forms of sarcoma is unknown, the only way to prevent some sarcomas is to avoid exposure to the risk factors above (though this isn’t always possible). Currently, there’s no proven way to prevent soft tissue sarcoma.

There are three main ways that cancer spreads in the body. It can spread through:

  • Cancer invades nearby normal tissue.
  • The lymph system. Cancer invades the lymph system and travels through the lymph vessels to other parts of the body.
  • Cancer invades veins and capillaries and travels through the bloodstream to other parts of the body.

The original tumor is called the primary tumor. When cancer cells break away from it and travel to other places in the body, a secondary tumor can form. The name for this spreading process is metastasis.

The secondary tumor is the same kind of cancer as the primary tumor. For instance, if sarcoma cells travel to the lungs, it is considered metastatic sarcoma. It is not lung cancer.

As you and your doctor explore the treatment options, make sure you find out the answers to the following:

  • What are my treatment choices? Which do you recommend for me and why?
  • Will I have more than one kind of treatment?
  • What are the expected benefits of each type of treatment?
  • What are the risks and possible side effects of each treatment? What can we do to control the side effects?
  • What can I do to prepare for treatment?
  • Will I need to stay in the hospital? If so, for how long?

You may want to ask these questions before the doctor takes a sample of tissue:

  • Which procedure do you recommend?
  • How will the tissue be removed?
  • Will I have to stay in the hospital? If so, for how long?
  • Will I have to do anything to prepare for it?
  • How long will it take?
  • Will I be awake?
  • Are there any risks?
  • How long will it take me to recover?
  • How soon will I know the results? Who will explain them to me?
  • What is the treatment likely to cost? Will my insurance cover the cost?
  • How will treatment affect my normal activities?
  • Would a clinical trial be right for me?
  • How often should I have checkups after treatment?
  • What are the chances my cancer will come back after this treatment?
  • What will we do if the cancer comes back or the treatment doesn’t work?
  • Will I lose my hair?
  • Will you have to remove lymph nodes?
  • Will it be painful?
  • Will there be scars?
  • What are the long-term and short-term side effects of treatment and how can I manage them?

Your immune system plays a big part in your fight against cancer. It is essential to stay as healthy as possible while undergoing cancer treatment. Here are some tips:

  • Practice .
  • Wash your hands often, and ask your family, healthcare providers and visitors to do the same.
  • Bathe every day with warm water and gentle soap.
  • Ask your doctor if you should get a flu vaccine or the COVID-19 vaccine.
  • Wear a mask when in public and around other people.
  • Brush your teeth after meals with a soft toothbrush.
  • Do your best to avoid cuts. Wear gloves when cleaning or gardening.
  • If you get a small cut, wash it with soap and water, apply antibiotic cream, and put a bandage over it.
  • Contact your doctor immediately if you suspect you have an infection, particularly if you have a fever.
  • Be physically active.
  • Maintain a healthy weight.
  • Don’t use tobacco products.
  • Protect your skin from the sun with sunscreen, sun-protective clothing and a wide-brimmed hat.
  • Eat plenty of vegetables and fruit.
  • Limit or avoid drinking alcohol.

Do your best to evaluate your financial situation as soon as possible. You will not want to deal with financial stress in the middle of treatment when you may not feel well. A social worker can help with this process. If you don’t currently have a social worker, ask your doctor or nurse for a recommendation

It’s important to plan ahead as much as possible when beginning cancer treatment. Ask your nurse navigator or social worker if they can recommend resources to help with the logistics of treatment.

With Your Healthcare Team

When going through cancer treatment, your healthcare team is very important. Your healthcare team may include your oncologists, surgeon, nurse navigator, dietitian, social worker or other medical professionals. Every member plays an important role. Use the tips below for talking with your healthcare team:

  • Establish your main point of contact.
    • Your main point of contact will probably be a nurse navigator, but it may be another member of your healthcare team. Find out who you should contact first with questions.
  • Don’t be afraid to ask questions.
    • Be open and honest with your healthcare team about your physical and emotional well-being.
    • Cancer is usually not a medical emergency. There is time to ask your healthcare team any questions you may have and consider your treatment options.
    • Write down your questions before your appointments. Take a pen and paper to write down the answers.
    • If you develop any new problems or symptoms during treatment, tell your healthcare team immediately. You are not complaining. This is valuable information for your doctors.
    • Do not change your diet, start an exercise program or take any new medications, including vitamins and supplements, during treatment without talking to your healthcare team first.

With Your Caregiver

Your primary caregiver may be with you when you receive your diagnosis. Your primary caregiver may be your spouse, partner, adult child, parent or friend. This person may come with you to appointments, take care of you after surgery or treatment and support you throughout your cancer journey. Here are some tips for communicating with your caregiver:

  • Everyone reacts to the news of cancer differently. You may feel upset, shocked or angry. It may take you some time to process the information. Your caregiver may react the same way you do or your caregiver may not. Even if they don’t react the same way you do, it doesn’t mean that they don’t care deeply.
  • Establish your role and your caregiver’s roles early. For example, will your caregiver be the one scheduling most of your appointments or do you prefer to take an active role? Find what works best for you and your caregiver.
  • Be open with each other about how you both feel. Overly positive attitudes may hinder honest communication. It’s okay to be upset.
  • Encourage your caregiver to take time to care for their own physical and emotional well-being. Being a caregiver comes with its own hardships.
  • If your primary caregiver is your spouse or partner, your intimate and physical relationship may change. Cancer treatment and side effects can potentially affect your libido and sexual function. If you have questions or concerns about sexuality and intimacy during and after cancer treatment, talk to your nurse, doctor or social worker.

With Your Children

Children are very perceptive, no matter their age. While you may wish to protect your children by not telling them about your cancer diagnosis, even young children may be able to tell that something is wrong. Not knowing what is wrong may cause them more stress and anxiety.

Here are some tips to talk to your children and teens about your cancer diagnosis:

  • Wait until your emotions are under control and decide what to say ahead of time.
  • Tell the truth and answer questions honestly. Depending on your children’s ages, it may not be appropriate to give them all the details, but do be truthful.
  • Let them know what to expect. For example, tell them that after surgery, you will need a lot of rest and may need to stay in the hospital. If your chemotherapy may cause you to lose your hair, let them know. Keep your children in the loop as much as possible.
  • Explain to your children, especially younger children, that they cannot “catch” cancer.
  • Let your children know that it is okay to cry or be upset. This may be especially important for your teens to hear.
  • Tell teachers, babysitters and others with responsibilities with and around your kids about your diagnosis in case they see behavior changes you may need to know about.
  • Maintain normal schedules as much as possible.
  • Let your kids help. Allow them to help with chores and let them know that their help is important. Teens may want to take an active caregiver role. Let them do so, at appropriate levels.
  • Look for support groups in your area. Many places offer support groups for children and teens whose parents have a cancer diagnosis.
  • Know when to seek professional help. If your child begins to demonstrate unusual behavior such as angry outbursts, nightmares or poor grades in school, ask your healthcare team for a recommendation for a counselor.

With Family and Friends

You may choose to keep your cancer journey private or you may choose to share your story with others. The choice is yours. Remember, when family, friends, coworkers or other acquaintances ask about your diagnosis, they are genuinely concerned about your well-being. You can share with them as much or as little information as you like. These suggestions may help you talk about your diagnosis:

  • Decide how much information you want to share before you start telling people about your diagnosis.
  • If you chose to keep your journey private, let people know that you appreciate their concern, but you hope they respect your privacy.
  • Choose someone close to you, like your caregiver, to spread the word about updates and treatment progress. After a long day of treatment, you may not feel like calling and texting people, but your friends and family will probably want to know how you are.
  • If you want to share your story, consider starting an email chain or a Facebook group. This way, you can update everyone with one message instead of answering a lot of emails and phone calls.
  • You can also create your own private website at MyLifeLine.
  • When people offer to help, let them. Your family and friends could cook dinner, drive you to an appointment, help clean your home, run errands or babysit.
  • If you lose your hair due to treatment or have visible surgical scars, strangers may ask about your diagnosis. Have a response prepared. Again, you may share as little or as much as you like.