This page was reviewed under our medical and editorial policy by
Maurie Markman, MD, President, Medicine & Science
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 vary patient to patient, depending upon the stage, grade, type and location of the tumor.
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.
The most common soft tissue sarcoma types are listed below, along with their tissues of origin.
Angiosarcoma forms in the inner lining of blood and lymph vessels. It's a rare cancer type, representing between 1 percent and 2 percent of all sarcoma diagnoses.
DFSP forms in the tissue under the skin, and is typically found in the trunk or limbs of young adult patients. DFSP is relatively uncommon, representing between 1 percent and 6 percent of all soft tissue sarcomas.
Epithelioid sarcoma is typically found in the hands or feet of young adults, appearing as small nodules. This type of cancer is very rare, representing less than 1 percent of all soft tissue sarcomas.
Ewing sarcoma is considered a type of bone sarcoma, but one-third of all Ewing tumors develop in the soft tissues and are categorized as extraosseous (outside of the bone) Ewing’s tumors. This type of cancer is most often diagnosed in teenagers, but it can also affect adults and younger children. Ewing tumors represent about 1 percent of all childhood cancers.
Fibrosarcoma forms in the fibrocytes, the cells that make up the fibrous tissue that envelops 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 outside the digestive tract can also occur. GISTs are rare, representing less than 1 percent of tumors that form in the gastrointestinal tract.
Kaposi sarcoma is a cancer of the blood vessels or lymph system and produces 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 less than 20 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 liposarcoma diagnosis so the care team may develop a treatment plan for that specific type or subtype.
An undifferentiated pleomorphic sarcoma, also referred to as a malignant fibrous histiocytoma, forms in the fibrous tissue, most commonly in the legs, but it may also develop in the abdomen. This is the second most-commonly diagnosed soft tissue sarcoma, after leiomyosarcoma.
Also referred to as neurofibrosarcoma, a malignant peripheral nerve sheath tumor is a type of soft tissue sarcoma that develops in peripheral nerves. This type of cancer represents between 5 percent and 10 percent of all soft tissue sarcoma diagnoses.
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.
Also called synovial cell sarcoma, this type of tumor most commonly forms in the areas around large joints. Synovial sarcomas represent between 5 percent and 10 percent of all soft tissue sarcomas.
Next topic: What are the stages of soft tissue sarcoma?