Desmoid Tumor

Desmoid tumors are thought to develop from the fibrous tissue that forms tendons and ligaments, most often in the arms, legs and abdomen (they can also occur the head and neck). When these tumors occur outside the abdomen, they are called aggressive fibromatosis. Desmoid tumors are a form of non-metastasizing, intermediate (locally aggressive) neoplasm that can adhere to and intertwine with surrounding structures and organs, making them difficult to control.

The exact cause of Desmoid tumors and fibromatosis is unknown, though some occur in patients with a history of polyposis coli, a hereditary colon cancer syndrome. Because Desmoid tumors affect tissue that is elastic and easily moved, these tumors may exist for a long time before being discovered, growing larger and pushing aside surrounding tissue. The most common symptoms of Desmoid tumors include:

• Painless swelling or a lump
• Pain or soreness caused by compressed nerves or muscles
• Pain and obstruction of the bowel
• Limp or other difficulty using the legs, feet, arms or hands

In addition to a complete medical and physical examination, the most conclusive diagnostic procedure for Desmoid tumor is a biopsy, a tissue sample from the tumor taken through a simple surgical procedure.

Treatment depends on the extent of the problem and your child's overall health and medical history.

Treatment your doctor recommends may include:

• Surgery: Surgical removal of your child's tumor by a surgeon is the primary treatment; and because Desmoid tumor rarely metastasizes, surgery alone is often the only treatment. However, Desmoid tumors often return, so sometimes more than one surgery is needed.
• Anti-inflammatory medication: Given to help manage pain and swelling. In some cases these medications will cause the tumor to slowly shrink.
• Radiation therapy: Using high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors; radiation is used alone or in conjunction with surgery.
• Hormone therapy: Because some hormones seem to increase the growth of Desmoid tumors, anti-hormonal medications are sometimes used in treatment.
• Chemotherapy: If surgeons are unable to remove your child's tumor because of the size or location, researchers are testing some types of chemotherapy to reduce the tumor.

The prognosis for patients with Desmoid tumors depends on how aggressively the tumors grow and whether they compress organs such as the intestines, kidneys, lungs, or blood vessels. More aggressive Desmoid tumors can be fatal.

Patients with Desmoid tumors and fibromatosis are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through the Bone and Soft Tissue Tumors Program.