Desmoid Sarcoma

Desmoid tumors develop in the tissues that form tendons and ligaments, usually in the arms, legs or abdomen and sometimes in the chest. These tumors, also called aggressive fibromatosis, are a type of soft tissue sarcoma and are considered benign because they typically don’t spread to other parts of the body. They may invade nearby tissues and organs, however, and can be difficult to control.


Although we don’t yet know the cause of these tumors, researchers have found that they tend to occur in children who have a family history of polyposis coli, a hereditary colon cancer. Treatments include surgery, chemotherapy and radiation, depending on the location of the tumor and age.

Distinct Clinical Features

Desmoid tumors usually manifest themselves as a painless or slightly painful mass (lump). Desmoid tumors can develop virtually at any body site. Superficial desmoids tend to be less aggressive than deep desmoids (abdominal, extra-abdominal, mesenteric). Desmoids inside the abdomen can cause gastrointestinal symptoms: change in bowel habits, pain, or rectal bleeding. They can invade the large mesenteric vessels and the vena cava. Desmoid tumors may have multiple sites of origin on an extremity, but they rarely occur at different regions in the same patient.


Desmoid tumors can be treated with surgery, radiation, hormone therapy, and medications such as nonsteroidal anti-inflammatory drugs (NSAIDs) and drugs under investigation, such as imatinib. Often a combination of these treatments offers the best chance of success.