A sarcoma is a rare type of malignant (cancerous) tumor that develops in bone and connective tissue, such as fat, muscle, blood vessels, nerves and the tissue that surrounds bones and joints. Symptoms depend on the tumor’s size and location. Treatments include surgery, radiation, chemotherapy, targeted therapy and immunotherapy.
A sarcoma is a malignant (cancerous) tumor that develops in bone and/or soft tissue. Soft tissues support other body structures and include:
Sarcomas are rare, making up only 1% of all adult cancer diagnoses and about 15% of childhood cancer diagnoses. Approximately 16,000 sarcomas are diagnosed in the United States each year (around 4,000 bone sarcomas and approximately 13,000 soft tissue sarcomas).
Sarcomas can form in various parts of your body — anywhere from your head to your toes:
Sarcoma refers to a large group of cancers in bone and/or soft tissue. There are more than 70 known subtypes of sarcoma.
Primary bone sarcoma is cancer that starts in the bone. More than one-third of bone sarcomas are diagnosed in people younger than 35 years old. Many are diagnosed in children.
Types of primary bone sarcoma include:
Unlike primary bone cancer, metastatic bone cancer starts in another location (such as an organ) and travels to the bone. This cancer comes in many forms, often traveling from your thyroid, lung, kidney, breast or prostate.
When cancer from a distant organ spreads to your skeleton, it can create structural problems in your bones that may cause pain and reduce functioning.
Soft tissue sarcomas begin in the muscle or other connective tissues of your body. Unlike bone sarcomas, most soft tissue sarcomas occur in adults. Certain types of sarcoma, such as rhabdomyosarcoma, are found mostly in children.
Types of soft tissue sarcomas include:
Sarcoma affects both children and adults. Generally, soft tissue sarcoma occurs more frequently in adults. Bone sarcoma diagnoses occur more often in children, teens and people older than 65 years. Bone sarcoma occurs more frequently in people assigned male at birth and people who are Black or Hispanic.
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Sarcomas form when immature bone or soft tissue cells have changes to their DNA, and they develop into cancer cells that grow in an unregulated way. They may eventually form a mass or tumor that can invade nearby healthy tissues. Left untreated, the cancer can travel via your bloodstream or lymphatic system from the primary location where it formed to other organs (metastasis). Metastasized cancer is challenging to treat.
As with other forms of cancer, researchers don’t know what causes a healthy cell to become sarcoma.
Certain factors may raise your risk of developing sarcoma. Risk factors include:
Symptoms vary depending on the tumor’s location. For example, some sarcomas may not cause noticeable symptoms in the early stages. Some sarcomas may feel like a painless lump under your skin, while others don’t cause pain until they grow large enough to press on an organ.
Other sarcomas can cause long-lasting bone pain or swelling in your arm or leg that worsens at night. These changes may limit your movement.
Symptoms may include:
Sarcoma shares symptoms with many other conditions. Your provider can help determine whether your symptoms are related to sarcoma or another disease or disorder.
Your healthcare provider will begin with a thorough history and physical examination. They may perform special tests on a tissue sample obtained from a biopsy to diagnose the exact type of sarcoma you have.
They may use any of the following tests when making a diagnosis:
Cancer staging is used to better understand how serious a sarcoma is and what treatments may work best. Providers classify most sarcoma based on the TNM classification system:
Your provider may also determine what grade (G) your cancer is. The grade provides information on the appearance of the cancer cells, including how much they resemble normal cells. Generally, the more normal the cancer cells appear, the better your prognosis.
Providers use this information to stage your sarcoma. They assign a number (1 through 4) to your stage. The higher the number, the more the cancer has grown locally or spread throughout your body.
There are different staging criteria for different types of sarcoma. Ask your provider about your cancer stage, including what this means for your treatment and prognosis.
Your care team may include:
Your treatment will depend on various factors, including:
Your provider will work to remove all cancer cells while sparing as much healthy tissue as possible. They’ll perform a wide local excision, while leaving a margin of healthy (“negative”) tissue at the edges. This approach removes the tumor without leaving any microscopic disease.
Rebuilding the affected area is also essential. Limb salvage (saving/preserving an arm or a leg) may involve removal of the tumor and, if needed, metal replacements for joint reconstruction. It may include replacing the bone removed during surgery with bone taken from another part of your body or elsewhere as needed. In some instances, the safest and best option is to amputate the affected limb, which can be an especially difficult decision.
Radiation therapy uses high-energy X-rays to kill cancer cells while minimizing damage to healthy cells. Radiation therapy may be internal (placed in your body) or external (delivered by a machine outside your body). Radiation uses advanced techniques so that radiation is directed toward the cancer while sparing healthy areas.
Internal radiation, also called brachytherapy, can be used to treat sarcoma that hasn’t spread. Brachytherapy can be delivered in two ways: intra-operative radiotherapy (during surgery) and interstitial brachytherapy, which is delivered through a series of catheters (plastic tubes) after surgery.
Radiation can also treat sarcoma that's spread (metastatic disease).
Chemotherapy uses drugs that kill or slow the growth of rapidly multiplying cancer cells. These medications are given intravenously (through a needle into a blood vessel) or orally. You may receive chemotherapy before surgery to shrink the tumor or after surgery. In some cases, chemotherapy is given with radiation.
Targeted therapy attacks weaknesses in cancer cells without harming healthy cells in the process. Targeted therapy only works on certain types of cancer cells with the weaknesses it’s designed to target. Your provider may recommend targeted therapy as a standalone treatment or in combination with other treatments.
Immunotherapy strengthens your immune system so that it’s better able to identify and fight cancer cells. Cancer cells have characteristics that help them hide from your immune system or deactivate your immune response. These features allow cancer cells to multiply and thrive in your body. Immunotherapy can undo these defenses so that your body can fight cancer cells.
Sometimes sarcomas can be heated or frozen to destroy the mass. An interventional radiologist does this procedure using guided imaging (for example, using a CT scan to guide the placement of a probe to freeze tissue).
Palliative care is specialized medical care for people with serious illnesses, including cancer. Palliative care professionals include doctors, nurses, social workers and nutritionists, among others. They can help with symptom relief and help you manage treatment side effects. Your palliative care team can help you navigate your cancer diagnosis, ensuring your quality of life is always a top priority.
Your healthcare provider may also recommend you take part in a clinical trial. A clinical trial is a study that tests the safety and effectiveness of new cancer treatments. New treatments for sarcoma are always in process. A clinical trial may be an option for you, depending on your diagnosis.
The five-year survival rate of soft tissue sarcoma ranges from 15% for metastasized cancer (cancer that has spread to other parts of your body) to 81% for cancer that hasn’t spread. The five-year survival rate for osteosarcoma (the most common type of bone cancer) ranges from 26% for metastasized cancer to 77% for cancer that hasn’t spread.
These numbers miss a lot of specifics that are essential for understanding your prognosis. Your outcomes depend on the type of sarcoma, its location, your general health, how you respond to treatment, etc. Your provider is your best resource for understanding your likely outcomes based on your unique case.
Schedule an appointment with your healthcare provider if you have a mass — whether it’s painful or not — or a mass that’s larger than a golf ball (around 5 centimeters). Contact your provider if you or your child have bone pain unrelated to an injury that doesn’t improve on its own.
A note from Cleveland Clinic
Sarcoma isn’t just one type of cancer. Instead, it refers to multiple types of growths that can develop in your bones or soft tissue. Symptoms vary depending on where your tumor’s located. If you’re diagnosed with sarcoma, consider that your treatment options and prognosis depend on multiple factors your healthcare provider can explain to you. Treatment options and likely outcomes are as varied as sarcomas are.
Last reviewed by a Cleveland Clinic medical professional on 05/27/2022.
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