Soft Tissue Sarcoma

“Soft tissue sarcoma“ is an overarching term for rare, cancerous tumors in your soft tissues. You have soft tissues everywhere in your body, including your:

• Blood vessels
• Body fat
• Ligaments
• Lymphatic vessels
• Muscle
• Nerves
• Tendons

Soft tissue sarcoma typically affects your arms, legs, chest and the back of your belly (retroperitoneum). Adults are more likely than children to have a type of soft tissue sarcoma.

Types of soft tissue sarcoma

There are more than 70 different types of soft tissue sarcoma. Some types, like liposarcoma, have several subtypes. Types of soft tissue sarcoma are:

• Angiosarcoma: This disease forms in the inner lining of your blood cells and lymphatic vessels. It’s a very aggressive type of soft tissue sarcoma.
• Desmoplastic small round cell tumors: These are tumors in the lining on the inside of your abdomen and pelvis.
• Gastrointestinal stromal tumors (GISTs): This type of soft tissue tumor develops in your digestive system.
• Leiomyosarcoma: This disease affects your smooth muscle. This type of tumor often develops in your belly.
• Liposarcoma: This tumor can develop in your legs or your retroperitoneum.
• Malignant peripheral nerve sheath tumor: These are tumors that form in layers of tissue protecting the nerves in your peripheral nervous system.
• Malignant schwannomas: These are slow-growing tumors. They can affect nerves in your arms, legs or your lower back.
• Myxofibrosarcoma: This soft tissue tumor may develop in fat or cartilage that’s right under your skin.
• Rhabdomyosarcoma: These tumors affect muscles in your arms, legs, head, neck and belly. Children and teenagers are more likely to have this cancer than adults.
• Synovial sarcoma: This type of tumor may form in your joints, belly, arms, legs or lungs.
• Undifferentiated pleomorphic sarcoma: This is the most common type of soft tissue sarcoma. It typically forms in your arms and legs.

What are the symptoms of soft tissue sarcoma?

Most soft tissue sarcomas grow very slowly. You can have one for months or years before you have symptoms. You should contact a healthcare provider if you have:

• A new lump or a fast-growing lump anywhere on your body
• A lump that starts to hurt
• Belly pain that gets worse
• Blood in your poop or poop that appears black
• Nausea and vomiting
• Unexplained weight loss

Soft tissue sarcoma causes

Experts don’t know the exact cause. Genetic mutations cause some soft tissue sarcomas. There are also inherited disorders and activities that increase your risk of having this condition. For example, some inherited disorders can increase your risk, including:

• Gardner syndrome
• Hereditary retinoblastoma
• Li Fraumeni syndrome
• Lynch syndrome
• Maffucci syndrome
• Neurofibromatosis type 1 (von Recklinghausen disease)

Other risk factors include:

• Receiving radiation therapy
• Experiencing lymphedema in your arms or legs for a long time
• Having exposure to chemicals, including thorium dioxide, arsenic or vinyl chloride

How doctors diagnose soft tissue sarcoma

A healthcare provider will ask about your symptoms. They’ll examine any unusual lumps or bumps that they can see and feel. They’ll do a physical exam to look for other symptoms. They may do the following imaging tests:

• X-ray
• CT scan
• MRI
• PET scan

Your provider may refer you to a surgical oncologist if imaging tests detect cancer. Your surgical oncologist may do a biopsy to get a small amount of tissue.

A medical pathologist will examine the tissue under a microscope. They may do several tests to identify the sarcoma type, stage and grade. Tumor grade is how pathologists find out if a tumor is likely to grow slowly, fast or spread.

What are the stages?

Healthcare providers use cancer staging systems to develop treatment plans and prognoses (what you can expect if you have soft tissue sarcoma). They consider the following factors in staging soft tissue sarcoma:

• Tumor size and location
• If the tumor is spreading to other areas of your body
• Tumor grade (this is how pathologists determine if a tumor is likely to grow and/or spread)

How is soft tissue sarcoma treated?

The most common treatment is surgery to remove a cancerous tumor. Your surgical oncologist may work with a medical oncologist. That provider may do additional treatments like:

• Chemotherapy
• Immunotherapy
• Targeted therapy
• Radiation therapy

You may want to consider participating in a clinical trial. Soft tissue sarcoma is a rare kind of cancer. Researchers are investigating new ways to treat it. Participating in a clinical trial may be a way to benefit from newer treatments. Ask your provider if seeking a clinical trial makes sense in your situation.

Is there a cure?

Surgery that removes soft tissue sarcoma before it spreads may cure the disease. But soft tissue sarcoma can come back after surgery and other kinds of treatments.

When should I seek care?

You should contact your healthcare team if you experience the following symptoms after your surgery:

• An opening in the incision line (the line may get deeper, longer or wider)
• Fever (greater than 101 degrees Fahrenheit or 38.4 degrees Celsius)
• Pain when you touch the wound or the area around the incision
• Skin discoloration that goes beyond the edge of the incision
• Thick, cloudy, white- or cream-colored discharge from the incision (cut)

And talk to your care team if treatment side effects are more severe than you expect. They may recommend palliative care. This is specialized care. Palliative care providers can work with you to ease treatment side effects.

Will I need follow-up care?

Yes. Some types of soft tissue sarcoma come back. Your healthcare provider may recommend regular follow-up tests. They may do these tests for the first two to three years after you finish treatment.

What is the life expectancy of someone with soft tissue sarcoma?

Life expectancy is how long you’ll live with soft tissue sarcoma. Your care team can’t predict that. But they may offer to explain soft tissue sarcoma survival rates.

Cancer survival rates are estimates based on the experiences of people who have cancer. The estimates are presented as the percentage of people who are alive five years after their cancer diagnosis.

The National Cancer Institute tracks cancer survival rates based on how far cancer has spread at diagnosis. Here are estimated survival rates for people who have soft tissue sarcoma: