• Desmoid Tumors and Fibromatosis in Children

    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. These benign tumors do not spread, but they can adhere to and intertwine with surrounding structures and organs, making them difficult to control.

    Desmoid Tumor Treatment at Dana-Farber/Boston Children's

    Patients with Desmoid tumors and fibromatosis are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through the Bone & Soft Tissue Tumor Program. Continue reading to learn more about Desmoid tumors or visit the Bone & Soft Tissue Tumor Program to learn about our expertise.

    Symptoms & Diagnosis

    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 physical examination, the most conclusive diagnostic test for Desmoid tumor is a biopsy to study a sample of tissue taken through a simple surgical procedure. After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care Options

    Since Desmoid tumors and fibromatosis do not spread, surgery is often the only treatment required. Because these tumors often return, additional operations may be needed. Other treatments include:

    • Anti-inflammatory medications to manage pain and swelling and which, in some cases, may shrink the tumor
    • Chemotherapy usually low doses  if the surgery is unable to remove the tumor because of its size or location
    • Direct ablation of the tumor with freezing (cryotherapy) or with heat (radiofrequency ablation). 

    Progressive or Recurrent Disease

    Desmoid tumors have very high local recurrence rate (close to 70%), and multiple treatment are usually needed. 

    Long-term Outlook

    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.
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