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The information on this page was reviewed and approved by
Maurie Markman, MD, President, Medicine & Science at CTCA.

This page was updated on March 1, 2021.

About soft tissue sarcoma

Soft tissue sarcomas may develop in any tissue that connects, supports or surrounds other structures and organs in the body. Soft tissues sarcomas may develop in the muscles, fascia (the tough membrane surrounding muscles), tendons, fat, blood vessels, nerves and synovial tissues (connective tissue that makes up the membranes surrounding joints).

Soft tissue sarcomas are rare in adults and account for less than 1 percent of all new cases of cancer. The American Cancer Society estimates that about 13,460 new cases of soft tissue sarcoma will be diagnosed in 2021 (7,720 cases in males and 5,740 cases in females). Sarcomas may be found almost anywhere in the soft tissues of the body.

According to the National Cancer Institute, about 50 percent of soft tissue sarcoma cases occur in the arms and legs, 40 percent occur in the back and chest, and 10 percent occur in the head and neck.

What causes soft tissue sarcoma?

Some common risk factors for soft tissue sarcoma, also called adult soft tissue sarcoma, include:

  • Hereditary conditions, including familial adenomatous polyposis (FAP or Gardner's Syndrome), retinoblastoma, neurofibromatosis type 1 (von Recklinghausen disease or NF1), tuberous sclerosis, Werner syndrome and Li-Fraumeni syndrome
  • Chemical exposure, including to vinyl chloride and dioxin
  • Previous radiation treatment
  • Weakened immune system, including from the human immunodeficiency virus (HIV)

Who gets soft tissue sarcoma?

While soft tissue sarcoma can occur in people of any age, it is more commonly found in adults over the age of 50.

Soft tissue sarcoma types

There are 50 different types of soft tissue sarcoma. Soft tissue tumors are generally named for the type of connective tissue in which they form. However, as researchers have learned more about this rare cancer, many names have changed over time. This type of cancer is typically described as low grade, mid grade or high grade, depending on the size of the tumor.

The following are the most common types of soft tissue sarcoma:

  • Angiosarcoma, forming in blood and lymph vessels
  • Dermatofibrosarcoma, commonly found in the trunk or limbs and forming in the tissue under the skin
  • Epithelioid sarcoma, appearing as small nodules typically found in the hands or feet of young adults
  • Ewing sarcoma, considered a type of bone sarcoma, though a third are categorized as extraosseous Ewing tumors developing in the soft tissues outside of the bone
  • Fibrosarcoma, forming in fibrocytes (cells that make up the fibrous tissue that envelops muscles, tendons and ligaments)
  • Gastrointestinal stromal tumors (GISTs), typically forming in the walls of the digestive system
  • Kaposi sarcoma, considered a cancer of the blood vessels or lymph system, producing reddish or purple lesions on the skin and commonly associated with HIV
  • Leiomyosarcoma, forming in large blood vessels and the pelvic area, including the uterus, and to a lesser extent in extremities such as the thighs
  • Liposarcomas, typically forming in deep layers of soft, fatty tissue and often confused with lipomas, which appear in more superficial soft tissue layers
  • Malignant fibrous histiocytoma (also called pleomorphic undifferentiated sarcoma), typically forming in the fibrous tissue of the legs but also occurring in the abdomen
  • Neurofibrosarcoma (also called malignant peripheral nerve sheath tumor), forming in the peripheral nerves
  • Rhabdomyosarcoma (RMS), an aggressive cancer forming in the body’s soft tissues, including muscles and connective tissue
  • Synovial sarcoma (also called synovial cell sarcoma), typically forming in areas around large joints

Soft tissue sarcoma symptoms

Soft tissue sarcoma symptoms may appear at a more advanced stage of the disease, or you might not notice any symptoms at all. Symptoms, if they are present, will vary depending on the type of sarcoma and its location.

Signs of soft tissue sarcoma may include:

  • A painless lump or mass where the tumor is growing, which may become painful if it is pressing on a nerve or muscle
  • Uncomfortable swelling around a tumor, especially when it is located in an arm or leg
  • Limited mobility as some tumors may restrict motion, such as those found in the hip, knee, shoulder or hands
  • Skin lesions

Other symptoms may have more to do with the parts of the body where a soft tissue sarcoma is located. For example, sarcomas in the abdomen may cause abdominal pain, vomiting or constipation, while sarcomas in the uterus may cause vaginal bleeding and/or abdominal pain.

Diagnosing soft tissue sarcoma

To distinguish soft tissue sarcoma from healthy tissue, cancer center pathologists use the following tools and tests:

  • Biopsy, including a needle biopsy or open surgical biopsy
  • Computed tomography scan (CT scan)
  • Magnetic resonance imaging (MRI)
  • Positron emission tomography (PET)/CT scan
  • Ultrasound
  • X-ray
  • Laparoscopic procedures

Soft tissue sarcoma treatments

Treatment options for cancer patients with soft tissue sarcoma may include:

  • Surgery (typically the primary treatment for soft tissue sarcoma) to locate and remove tumor cells
  • Chemotherapy
  • Radiation therapy
  • Targeted therapy, including so-called kinase inhibitors that target specific protein receptors that regulate cell growth and kill cancer cells