Types of soft tissue sarcoma

This page was reviewed under our medical and editorial policy by

Maurie Markman, MD, President, Medicine & Science at CTCA.

This page was updated on June 6, 2022.

There are 50 different types of soft tissue sarcomas, which can develop in many different locations and tissues within the body. Because of this great variability among soft tissue sarcomas, recommended treatment options will vary, depending upon the stage, grade, type and location of the tumor. Thus, developing an individualized treatment plan with your Cancer Treatment Centers of America® (CTCA) sarcoma care team is extremely important.

The various types of soft tissue sarcomas are generally named for the type of connective tissue in which they form. However, as researchers have learned more about these rare tumors, many of these names have changed over time.

Following are the most common soft tissue sarcoma types, as well as their tissues of origin:

Angiosarcoma forms in blood and lymph vessels.

Dermatofibrosarcoma forms in the tissue under the skin, commonly found in the trunk or limbs.

Epithelioid sarcoma is typically found in the hands or feet of young adults, appearing as small nodules.

Ewing’s sarcoma is considered a type of bone sarcoma, though one-third of all Ewing’s tumors develop in the soft tissues and are categorized as extraosseous (outside of the bone) Ewing’s tumors.

Fibrosarcoma forms in the fibrocytes, which are the cells that make up the fibrous tissue that envelopes muscles, tendons and ligaments. This type of soft tissue sarcoma is commonly found in the arms, legs or trunk, but can also be found deeper inside of the body.

Gastrointestinal stromal tumors (GISTs) develop in the walls of the digestive system, most commonly in the stomach. However, rare cases of GISTs arising from outside of the digestive tract can also occur.

Kaposi sarcoma is a cancer of the blood vessels or lymph system and is known for producing reddish or purple plaques on the skin. The most common form of Kaposi sarcoma is associated with infection by the human immunodeficiency virus (HIV), the virus that causes AIDS. The AIDS-related version of Kaposi sarcoma can be aggressive if it is not treated. It can form sores on the skin, spread to the lymph nodes and sometimes involve the gastrointestinal tract, lungs, heart and other organs. Treating HIV-infected patients with a so-called cocktail of highly active antiretroviral therapy (HAART) has dramatically lowered the U.S. incidence of Kaposi sarcoma. Today, about six in 1 million people are infected with the disease, about an eighth of the incidence rate of the early 1990s.

Leiomyosarcoma is an uncommon type of malignant tumor that grows from immature smooth muscle cells, accounting for between 10 percent and 20 percent of all soft tissue sarcomas. These tumors appear most often in large blood vessels and the pelvic area, including the uterus, and, to a lesser extent, in the extremities like the thighs. In its metastatic state, leiomyosarcoma most commonly spreads to the bloodstream, lung and liver. Age is considered a high risk factor for this disease. It most commonly affects people in their 70s. Symptoms vary depending on the location of the tumors. They frequently cause discomfort, swelling, blood clots and pain.

Liposarcomas account for 15 percent to 25 percent of the estimated 13,000 soft tissue sarcomas diagnosed each year. Most liposarcomas develop in deep layers of soft, fatty tissue and are often confused with lipomas, which appear in more superficial soft tissue layers. Liposarcomas are categorized by several types and subtypes, each with differing risk profiles. So, it’s important to get an accurate diagnosis of your liposarcoma in order to develop a treatment plan for that specific type or subtype.

Malignant fibrous histiocytoma forms in the fibrous tissue, most commonly in the legs, but may also occur in the abdomen. Also more recently known as pleomorphic undifferentiated sarcoma.

Neurofibrosarcoma, also known as a malignant peripheral nerve sheath tumor, is a type of soft tissue sarcoma develops in peripheral nerves.

Rhabdomyosarcoma (RMS) is an aggressive cancer that develops in the body’s soft tissues, including muscles and connective tissue. Rhabdomyosarcoma may occur at any age. It most often affects children and young adults, but its faster-growing types are more common in adults, who are also more likely to have the disease in hard-to-reach areas of the body, making it more difficult to treat. Tumors may occur throughout the body, including:

Synovial sarcoma, also called synovial cell sarcomas, is a type of tumor that most commonly forms in the areas around large joints.

Next topic: What are the stages of soft tissue sarcoma?

Expert cancer care

is one call away.
appointments in as little as 24 hrs.