What Is Sarcoma?

(sar·co·ma) n. a malignant tumor arising in tissue (as connective tissue, bone, cartilage, or striated muscle).

Sarcomas can start anywhere in the body but are most common in the arms, legs, abdomen and trunk. There are two main types, bone sarcomas and soft tissue sarcomas.

In 2014, approximately 15,000 cases of sarcoma were diagnosed in the United States (12,000 for soft tissue and 3,000 for bone) according to the National Cancer Institute with approximately 6,200 people expected to die from the disease. Twenty percent (20%) of all childhood cancers are sarcoma.

Despite the odds, there are many people who survive a sarcoma diagnosis.

Sarcoma is a rare cancer with more than 50 sub-types in two categories:

Soft-tissue sarcomas: develop in the soft tissues that surround, connect or support the body’s structure and organs. This includes muscles, joints, tendons, fat, blood vessels, nerves and tissues. They also can begin in the body’s organs. The most common soft tissue sarcomas in adults are malignant fibrous histiocytoma, liposarcoma, and leiomyosarcoma.

Bone sarcomas: arise in the bone. The most common types of primary bone cancers are osteosarcomas, chondrosarcomas and the Ewing’s family of tumors.

At any moment, there are more than 50,000 patients and their families affected with this disease.

Most sarcomas develop in people with no known risk factors so there is no known way to prevent them at this time. The best approach to early detection is to notify your health care professional of any unexplained lumps or growths. If you have any of the following problems, see a doctor right away:

  • A new lump or a lump that is growing anywhere on your body
  • Abdominal pain that is getting worse
  • Blood in your stool or vomit
  • Black, tarry stools

Since symptoms of soft tissue sarcomas often do not appear until the disease is advanced, only about 50% of soft tissue sarcomas are found in the early stages before they have spread. Because of their rarity and more than 50 sub-types, patients are strongly encouraged to visit a National Cancer Institute (NCI designated) or Sarcoma specialty center if sarcoma is suspected for final diagnosis and treatment.

Because treatment varies for the different sub types  your cancer care team should include a pathologist who can determine the exact type of sarcoma you have.  Options that may be included in your treatment plan include:

    • Surgery: primary treatment for soft tissue sarcoma
    • Radiation Therapy: may be given before, during, and after surgery
    • Chemotherapy: may be given before and/or after surgery
    • Targeted Therapy:  newer treatment that uses drugs or other substances to identify and attack cancer cells while doing little damage to normal cells
Type Origin
Alveolar Soft-part Sarcoma Occurs in the legs. Most commonly diagnosed in young adults.
Angiosarcoma blood or lymph vessels. This cancer is linked to radiation exposure and may start in the area originally treated with radiation for a different cancer.
Clear Cell Sarcoma Tendons of the arms or legs.
Desmoplastic Small Round Cell Tumor Abdomen. Often diagnosed in young adults.
Epithelioid Sarcoma In the tissue under the skin of hands; forearms;feet or lower legs.Often diagnosed in young adults.
Fibrosarcoma Tendons and ligaments (fibrous tissue)usually in the arms; legs; or trunk. This cancer is most common in patients ages 20 to 60 years old but can occur at any age.
Gastrointestinal Stromal Tumor (GIST) Digestive tract
Kaposi Sarcoma cells lining blood or lymph vessels. These tumors are more often seen in patients diagnosed with HIV or other suppressed immune systems (e.g. organ transplants).
Leiomyosarcoma Involuntary muscle (smooth muscle)such as that found in the uterus and digestive tract
Liposarcoma Fat tissue usually in the thighs; behind the knees or body cavities. Occur mostly in adults 50 to 65 years old.
Malignant Fibrous Histiocytoma (MFH) arms or legs. Most common in older adults.
Neurofibrosarcoma Peripheral-nerve sheaths in arm; legs; or trunk
Rhabdomyosarcoma Skeletal muscle; usually in arms or legs but can also occur in the head or neck area; vagina or bladder.
Spindle Cell Sarcoma Based on appearance of cells under a microscope (long and narrow). Not a specific diagnosis or type of cancer.
Synovial Sarcoma Cell of origin unknown. These tumors are often associated with the joints


Facts about Sarcoma*

  • Every day, 41 adults are diagnosed with sarcoma.
  • 1 in 150 families will have someone who is facing (or faced) sarcoma – when considering incidence over a lifetime.
  • More than half of sarcomas begin in an arm or leg.
  • There are more than 50 sub-types of soft tissue sarcoma.
  • Most soft tissue sarcomas develop in people who have no apparent risk factors.
  • The 5-year relative survival rate for both bone and soft tissue sarcoma is 65%.
  • 35% of Americans diagnosed with sarcoma do not survive.

To learn more about Adult Soft Tissue Sarcoma visit the EDDF Learning Center. 


*Information adapted from the American Cancer Society 


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