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About Childhood Desmoid Tumors

  • Desmoid tumors develop in the fibrous tissue that forms tendons and ligaments, most often in the arms, legs, and abdomen. They can also occur in the head and neck.

    Desmoid tumors that occur outside the abdomen are called aggressive fibromatosis. They're benign tumors because they do not spread. However, they can adhere to and intertwine with surrounding structures and organs, making them difficult to control.

    At Dana-Farber/Boston Children's Cancer and Blood Disorders Center, our doctors have deep expertise in diagnosing, treating, and caring for children with desmoid tumors. When you come to us for care, your child will be in the hands of renowned specialists in our Bone and Soft Tissue Tumors Program.

    Causes and Symptoms of Childhood Desmoid Tumors

    The exact cause of Desmoid tumors and fibromatosis is unknown. However, some occur in patients with a history of polyposis coli, a hereditary colon cancer syndrome.

    Desmoid tumors affect elastic and easily moved tissue, so they may unknowingly exist for some time before diagnosis.

    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

    How We Diagnose Childhood Desmoid Tumors

    Your doctor will start with a complete physical examination. Then, they will typically perform a biopsy to study a sample tissue to confirm the diagnosis. After we complete all tests, your doctor will be able to outline the best treatment options for your child's unique needs.

    How We Treat Childhood Desmoid Tumors

    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 in low doses, if the surgery is unable to remove the tumor because of its size or location
    • Direct tumor ablation (removal of the tissue) with cryotherapy (freezing) or radiofrequency ablation (heating)

    We will continue to care for your child after treatment through our comprehensive pediatric cancer survivorship programs. We provide everything from ongoing medical monitoring and care to psychosocial and nutritional support to ensure your child receives the best possible care.

    Long-term Outcomes for Children with Desmoid Tumors

    The prognosis for patients with Desmoid tumors depends on how aggressively the tumors grow and whether they compress the intestines, kidneys, lungs, or blood vessels. Your doctor will be able to discuss the prognosis based on your child's condition.

    Childhood Desmoid Tumor Treatment Team

    At Dana-Farber/Boston Children's, our team of pediatric cancer doctors works together to ensure that your child receives the most accurate, timely, and effective care possible. Learn more about the specialists in our Childhood Solid Tumor Center.