Sarcoma

Cancer begins when cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body).

About sarcoma

Both children and adults can develop a sarcoma. It can start in any part of the body, such as the bone or soft tissue. About 60% begin in an arm or leg, 30% start in the trunk or abdomen, and 10% are in the head or neck. Sarcoma is rare in adults, accounting for about 1% of all adult cancers. However, sarcoma in general represents about 15% of all cancers in children.

This section covers sarcoma of the soft tissues. Learn more about sarcoma that starts in a bone.

About soft-tissue sarcoma

Soft-tissue sarcomas (STS) are a group of cancers that begin in the tissues that support and connect the body. STS can occur almost anywhere in the body. The sarcoma cells resemble the cells that hold the body together, including fat cells, muscle, nerves, tendons, joints, blood vessels, or lymph vessels. When an STS is small, it can go unnoticed, since it does not usually cause problems early on. As an STS grows, it can cause problems with the body's normal activities.

Because there are several different types of STS, it is more of a family of related diseases rather than a single, specific disease. The specific types of sarcoma are often named according to the normal tissue cells they most closely resemble (see below). This is different from most other types of cancer, which are usually named by the part of the body where the cancer began.

Name of Sarcoma	Related Normal Tissue Type
Angiosarcoma	Blood or lymph vessels
Ewing Family of sarcomas (peripheral primitive neuroectodermal tumor, PNET)	Mesenchymal stem cells (cells that develop into connective tissue, blood vessels, and lymphatic tissue)
Fibrosarcoma	Fibrous tissue (tendons and ligaments)
Gastrointestinal stromal tumor (GIST)	Specialized neuromuscular cells of the digestive tract
Kaposi sarcoma	Blood vessels
Leiomyosarcoma	Smooth muscle
Liposarcoma	Fat tissue
Myxofibrosarcoma (myxoid malignant fibrous histiocytoma [MFH])	Connective tissue
Malignant peripheral nerve sheath tumor (MPNST, also known as neurofibrosarcoma)	Peripheral nerve
Pleomorphic sarcoma, not otherwise specified (NOS; often referred to in the past as malignant fibrous histiocytoma, MFH)	Undifferentiated connective tissue stem cells
Rhabdomyosarcoma	Skeletal muscle
Synovial sarcoma	Primitive mesenchymal stem cells

Grade is the term a pathologist (a doctor who specializes in interpreting laboratory tests and diagnosing disease) uses to describe how aggressive the sarcoma is likely to be, meaning how likely it will grow and spread to another part of the body. A low-grade tumor is usually less likely to spread quickly, and it often stays in the place where it started. A high-grade tumor is more likely to spread to other places, called metastasis. Learn more about grade in the Staging section.

Experts have found many types and subtypes of sarcomas. Pathologists are now trying to find new ways to quickly determine a tumor's subtype, as this helps determine treatment. Looking at a tumor's abnormal genetics may help determine its characteristics and predict which treatments will be most effective. For at least two types of sarcoma, GIST and dermatofibrosarcoma protuberans (DFSP), major advances have been made in a type of treatment called targeted therapy (see Treatment).

Find out more about basic cancer terms used in this section.

Looking for More of an Overview?

If you would like additional introductory information, explore these related items on Cancer.Net:

• Cancer.Net Patient Education Video: View a short video led by an ASCO expert in this type of cancer that provides basic information and areas of research.
• Cancer.Net En Español: Read about sarcoma in Spanish.

Or, choose “Next” (below, right) to continue reading this detailed section. To select a specific topic within this section, use the icon panel located on the right side of your screen.

This year, approximately 11,280 people (6,110 males and 5,170 females) will be diagnosed with soft-tissue sarcoma in the United States. An estimated 3,900 adults and children (2,050 males and 1,850 females) are expected to die of the disease this year.

The overall five-year relative survival rate (percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases), combining all stages and types of sarcoma, is approximately 51%. However, when the sarcoma is located in an arm or leg, the five-year survival rates are slightly higher for each stage.

How long a person lives with sarcoma depends on many factors, including the size of the tumor, where it is located, the type, the mitotic rate (how fast the tumor cells are growing and dividing), and whether it is superficial or deep (see Staging). If the sarcoma is diagnosed at an early stage and hasn't spread from where it started, treatment is very effective and many people can be cured. on the other hand, if the sarcoma has spread to other parts of the body, treatment can usually control the tumor, but it is not often curable.

Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of people with this type of cancer, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with sarcoma. Because the survival statistics are measured in five-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer. Learn more about understanding statistics.

Statistics adapted from the American Cancer Society's publication,Cancer Facts & Figures 2012.