Sarcoma

What is sarcoma?

A sarcoma is a malignant (cancerous) tumor that develops in bone and/or soft tissue. Soft tissues support other body structures and include:

• Muscle.
• Fat.
• Tendons.
• Cartilage.
• Ligaments.
• Blood vessels.
• Nerves.

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).

Where are most sarcomas found?

Sarcomas can form in various parts of your body — anywhere from your head to your toes:

• 40% occur in your lower extremities (legs, ankles, feet).
• 15% occur in your upper extremities (shoulders, arms, wrists, hands).
• 30% occur in your trunk/chest wall/abdomen/pelvis.
• 15% occur in your head and neck.

What are the types of sarcoma?

Sarcoma refers to a large group of cancers in bone and/or soft tissue. There are more than 70 known subtypes of sarcoma.

Bone sarcomas

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:

• Osteosarcoma (most common).
• Chondrosarcoma.
• Chordoma.
• Ewing’s sarcoma.
• Fibrosarcoma.

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

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:

• Angiosarcoma.
• Desmoplastic small round cell tumors.
• Gastrointestinal stromal tumor (GIST).
• Leiomyosarcoma.
• Liposarcoma.
• Malignant peripheral nerve sheath tumor.
• Malignant schwannoma.
• Myxofibrosarcoma.
• Rhabdomyosarcoma.
• Synovial sarcoma.
• Undifferentiated pleomorphic sarcoma.

Who is affected by sarcoma?

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.

What causes sarcoma?

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.

What are the risk factors associated with sarcoma?

Certain factors may raise your risk of developing sarcoma. Risk factors include:

• Exposure to chemicals: Exposure to arsenic and certain chemicals used to make plastics (vinyl chloride monomer), herbicides (phenoxyacetic acid) and wood preservatives (chlorophenols).
• Radiation: Exposure to high doses of radiation from previous cancer treatment.
• Lymphedema: Long-term swelling in your arms or legs.
• Genetic conditions: Certain inherited disorders and chromosome mutations, such as Gardner syndrome, Werner syndrome, von Hippel-Lindau disease, Gorlin syndrome, tuberous sclerosis, Li-Fraumeni syndrome, retinoblastoma and neurofibromatosis type 1.

What are the symptoms of sarcoma?

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:

• A new lump that may or may not hurt.
• Pain in an extremity (arm/leg) or abdomen/pelvis.
• Trouble moving your arm or leg (a limp or limited range of motion).
• Unexplained weight loss.
• Back pain.

Sarcoma shares symptoms with many other conditions. Your provider can help determine whether your symptoms are related to sarcoma or another disease or disorder.

How is sarcoma diagnosed?

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:

• X-ray: X-rays use small amounts of radiation to take pictures of bones and soft tissues inside your body.
• Computed tomography (CT) scan: A CT scan uses computers to combine many X-ray images into cross-sectional views of the inside of your body.
• Magnetic resonance imaging (MRI): An MRI uses large magnets, radio waves and a computer to create clear images of the inside of your body. Your provider may order an MRI to see more detailed pictures if an X-ray shows something abnormal.
• Bone scan: During a bone scan, your provider will inject a small amount of radioactive material into your body to identify bone disorders, such as bone sarcoma.
• PET scan: A PET scan uses a special glucose tracer that clings to cells using high amounts of glucose, like cancer cells. A PET scan shows parts of your body where glucose levels are unusually high, suggesting a tumor.
• Biopsy: During a biopsy, your provider removes tissue from your tumor and sends the sample to a lab. A specialist called a pathologist analyzes the tissue under a microscope to see if it’s a sarcoma. This analysis helps your provider understand what type of sarcoma you have and what treatments may work best.

What are the stages of sarcoma?

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:

• T: Describes the size and location of the primary tumor.
• N: Describes whether the cancer has spread to nearby lymph nodes.
• M: Describes whether the cancer has spread to other organs (metastasized).

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.

How are sarcomas treated?

Your care team may include:

• Surgeons.
• Radiologists.
• Geneticists.
• Medical oncologists (cancer specialists).
• Radiation oncologists.
• Pathologists.
• Pediatric specialists (for cancers in children).
• Psychologists.
• Social workers.

Your treatment will depend on various factors, including:

• The type of sarcoma.
• Its size and location.
• Your general health.
• Whether the sarcoma is newly diagnosed or returned (recurrent).

Surgery

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

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

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

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 (biologic therapy)

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.

Thermal ablation

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

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.

Clinical trials

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.

What is the survival rate of sarcoma?

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.

When should I see my healthcare provider?

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.