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About Childhood Fibrosarcoma

  • A fibrosarcoma is a malignant (cancerous) tumor that originates in the connective fibrous tissue found at the ends of bones of the arm or legs. It often spreads to other surrounding soft tissues. Soft tissues include fat, muscles, tendons (bands of fiber that connect bones to muscle), nerves, joint tissue, blood vessels, and other fibrous tissue.

    • Fibrosarcoma most commonly affects either a lower leg or arm.

    • There are two forms of fibrosarcoma:

      • Infantile or congenital fibrosarcoma: This type of tumor is the most common soft tissue sarcoma found in children under one year of age. It presents as a rapidly growing mass at birth or shortly after. This fibrosarcoma is usually slow-growing. It tends to be more benign than fibrosarcoma in older children, which behaves more like the type found in adults.

      • Adult form fibrosarcoma: The adult form of this disease can occur in older children and adolescents, roughly between 10 and 15 years old. It is more aggressive than the infantile form and generally involves more complex treatment.

    At Dana-Farber/Boston Children's Cancer and Blood Disorders Center, our pediatric surgical oncologists in the Bone and Soft Tissues Treatment Program have extensive experience treating babies and children with fibrosarcoma.

    Causes and Symptoms of Childhood Fibrosarcoma

    The exact cause of fibrosarcoma and other soft tissue tumors is not entirely understood. However, studies have indicated that genetic alterations may play a role. Researchers have studied a small number of families that contain several members from one generation who have developed soft tissue sarcomas.

    Limited studies have shown a possible link between soft tissue sarcomas and the development of other types of cancer. Researchers have found a chromosomal rearrangement in some fibrosarcomas.

    Each child may experience fibrosarcoma symptoms differently, depending on the size, location, and spread of the tumor.

    Fibrosarcoma symptoms may include:

    • A painless or tender mass in an extremity or trunk
    • Pain or soreness caused by suppressed nerves and muscles
    • Limping or other difficulty using legs, feet, arms hands

    Fibrosarcoma symptoms may resemble other conditions or medical problems. Always consult a physician for a diagnosis.

    How We Diagnose Childhood Fibrosarcoma

    We use several diagnostic procedures to determine the type of tumor and whether it has spread. Your child's doctor will perform a complete medical and physical examination. They may also order several different tests, including advanced imaging studies, biopsy, and bone scan.

    Once your doctor diagnoses fibrosarcoma, they will stage the tumor. This process specifies how far the tumor has spread from its original location. The cancer stage suggests which form of treatment is most appropriate and gives some indication of prognosis.

    A fibrosarcoma may be localized, meaning it has not spread beyond the bone where it arose or beyond nearby tissues. It can also be metastatic, meaning it has spread to lungs, bones other than the bone that the tumor originated in, or other organs or structures of the body. Tumors found during infancy are usually not metastatic.

    How We Treat Childhood Fibrosarcoma

    Surgery is the primary treatment for fibrosarcoma. Our goal is to remove the tumor entirely. We may use radiation therapy or chemotherapy in combination with surgery if needed. Treatment plans can vary depending on each child's situation and the extent of the tumor.

    Your child's treatment may include:

    • Surgery: We may perform the following surgeries depending on the location of the tumor.

      • Limb-salvage surgery: If the tumor is in the limb, we will take it out along with wide margins of healthy tissue surrounding the tumor to ensure the whole tumor is removed. The surgeon will maintain as much of the limb function as possible. Because limb-salvage surgery can leave a leg or an arm fragile and increase fracture risk, children need to avoid high-stress physical activities, such as bike riding, to prevent fractures.

      • Rotationplasty: This procedure is a partial amputation of the leg. It preserves the cancer-free lower leg, attaches it to the thighbone, and uses the ankle as a knee joint. One of the significant benefits of rotationplasty is that children can have a very active lifestyle, including playing high-impact sports.

      • Amputation: If we can't entirely remove the tumor because it involves the nerves and blood vessels, we may need to perform amputation surgery. If amputation is necessary, we will fit your child for a prosthesis following surgery.

      • Head or neck surgery: If the fibrosarcoma is in the head or neck, we will remove it with surgery.

    • Radiation therapy: This treatment can help stop the abnormal cells' growth in specific areas of the body. Radiation therapy uses high-energy rays from a specialized machine to damage or kill abnormal cells. Our doctors use precisely targeted and dosed radiation to kill cancer cells left behind after your child's surgery.

    • Chemotherapy: Chemotherapy medicines help stop abnormal cells' growth throughout the body. It is a systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to destroy or slow the growth of targeted cancer cells.

    After treatment, your child may receive rehabilitative and supportive care to help them prevent or treat side effects and get back to functioning in their daily lives. Our pediatric cancer survivorship programs also include patient and family education, psychosocial assessment, and genetic counseling.

    Long-term Outcomes for Children with Fibrosarcoma

    Each child's prognosis greatly depends on:

    • The extent of the disease
    • Size and location of the tumor
    • Presence or absence of metastasis
    • Tumor's response to therapy
    • Age and overall health of the child
    • Child's tolerance of specific medications, procedures, or therapies
    • New developments in treatment

    Childhood Fibrosarcoma Treatment Team

    The Dana-Farber/Boston Children's solid tumor treatment team includes childhood soft tissue tumor specialists who care for children with fibrosarcoma. Our doctors have the experience to get you the right diagnosis and the latest treatments.

    See a complete list of the specialists in our Childhood Solid Tumor Center.