Desmoid Tumors

What is a desmoid tumor?

A desmoid tumor is a growth that resembles scar tissue. However, it occurs inside the body and may be found anywhere within the connective tissues. Connective tissues provide strength and flexibility to bones, ligaments and muscles. A desmoid tumor is not usually considered cancerous.

Desmoid tumors are usually named for the areas where they grow:

• Abdominal desmoid tumors—in the walls of the abdomen (belly)
• Intra-abdominal desmoid tumors—in tissue connecting abdominal organs
• Extra-abdominal desmoid tumors—anywhere else in the body

How common are desmoid tumors?

Desmoid tumors account for 0.03 percent of all cancerous tumors. They occur most often in people with a family history of colorectal cancer, especially in those with a rare, inherited condition called familial adenomatous polyposis:

• In familial adenomatous polyposis, polyps (extra tissue) form in the large intestine and in the upper portion of the small intestine.
• Desmoid tumors occur in up to 20 percent of people with familial adenomatous polyposis of the colon.

Desmoid tumors in children are most commonly diagnosed between ages 15 and 16. Race and ethnic background do not seem to affect whether a person gets these tumors.

What are the symptoms of desmoid tumors?

Desmoid tumors develop in tissue such as tendons and ligaments. These tissues are flexible and easy for tumors to push aside as they grow. So these tumors are often present for a long time before they are found.

Symptoms vary depending on tumor size and location. Common symptoms include the following:

• A painless swelling or lump
• Pain or soreness caused by the tumor pressing on nerves or muscles
• Limping (leg tumors)
• Abdominal (belly) pain, constipation and other problems with the bowel being blocked
• Tingling or the feeling that an area of the body has “gone to sleep” (tumor pressing on a blood vessel or nerve)

How are desmoid tumors treated?

If your child has a desmoid tumor, treatment will depend on the patient’s:

• Medical history,
• Overall health,
• Age,
• Tumor size and expected growth, and
• Ability to withstand treatment.

Desmoid tumor treatment may include surgery, radiation therapy, chemotherapy and antihormonal therapy as well as medicines called nonsteroidal anti-inflammatory drugs (NSAIDs):

• Surgery — is the most common way to treat these tumors. They rarely spread (metastasize), so surgery is often the only treatment needed. However, if the tumor is not completely removed or returns, more surgery may be required.
• Radiation therapy — uses high-energy X-rays or other types of radiation to kill cancer cells or stop them from growing:
  • External radiation uses machines outside the body to deliver the X-ray dose.
  • Internal radiation uses needles, seeds, wires or catheters (tubes) to deliver the radiation directly into or close to the cancer.

Radiation therapy may be used alone or in addition to surgery.

• Chemotherapy (“chemo”) — uses powerful medicines to kill cancer cells or stop them from growing (dividing) and making more cancer cells.
  • Chemo may be injected into the bloodstream, so that it can travel throughout the body.
  • Some chemo may be given by mouth.
  • Combination therapy uses more than one type of chemo at a time.

Chemo may be used for desmoid tumors that cannot be removed safely because they are too close to blood vessels or other important structures.

• Antihormonal therapy — uses medicines that help block the growth of some kinds of desmoid tumors due to the actions of certain hormones (chemicals in the blood).
• Nonsteroidal anti-inflammatory drugs (NSAIDs) — may be prescribed to help manage pain and swelling related to desmoid tumors.

What are the survival rates for desmoid tumors?

Up to 30 percent of these tumors come back within 10 years after surgery. Fortunately, they are rarely fatal.

Why choose St. Jude for your child’s desmoid tumor treatment?

• St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.
• St. Jude has created more clinical trials for cancer than any other children’s hospital in the United States.
• The nurse-to-patient ratio at St. Jude is unmatched— averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.
• The expert skills and experience of St. Jude specialty surgeons can help improve patients’ chances for best outcomes.
• Your child will have access to a close-knit team of specialists that includes: surgeons; doctors and nurses who treat this cancer; doctors who specialize in radiation therapy, in hormones (endocrinologists), and in diagnosis (pathologists); experts in diagnostic imaging and nuclear medicine; dietitians; speech therapists; rehabilitation specialists (physical therapists, occupational therapists); child life specialists; psychologists; Quality of Life team members; experts who help manage and minimize the long-term or late effects of treatment; researchers; scientists; and many others.
• The medical team works closely with laboratory researchers to bring new treatments from the laboratory to the clinic.