• Fibrosarcoma in Children

    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, and then spreads to other surrounding soft tissues. It is a type of soft tissue tumor; 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 form of fibrosarcoma is usually slow-growing, and 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 in adolescents, roughly between the ages of 10 and 15. It is more aggressive than the infantile form and generally involves more complex treatment.

    Fibrosarcoma Treatment at Dana-Farber/Boston Children's

    Children with fibrosarcoma are treated at Dana-Farber/Boston Children's through our Bone and Soft Tissue Program. Our integrated pediatric oncology program offers the combined expertise of a leading cancer center and a world-renown children’s hospital.

    • We provide the full set of options that can be used to treat bone and soft tissue tumors, and our solid tumor treatment team can help you determine which option is best for your child.
    • Our pediatric oncologists have access to, and often lead, some of the most innovative clinical trials for soft tissue sarcomas, including fibrosarcoma.
    • Our surgeons have specialized expertise in complex surgeries, and our radiation oncologists have access to the newest radiation therapy techniques that may also be used in the care of a child with fibrosarcoma.

    What are fibrosarcoma causes and symptoms?

    Fibrosarcoma causes

    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. In addition, limited studies have shown a possible link between soft tissue sarcomas and the development of other types of cancer. A chromosomal rearrangement has been found in some fibrosarcomas.

    Fibrosarcoma symptoms

    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 is fibrosarcoma diagnosed?

    In addition to a complete medical history and physical examination of your child, diagnostic procedures for fibrosarcoma may include:

    • Multiple imaging studies of the tumor and sites of possible metastasis, such as:
      • x-rays – a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto filmX-rays are very useful in the diagnosis of bone tumors and frequently x-rays allow the physician to distinguish between benign and malignant tumors. An x-ray is usually the first diagnostic study and it often gives the doctor information regarding the need for further testing.
      • magnetic resonance imaging (MRI) – a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. This test is used to assess the size and extent of the mass and its relationship to surrounding muscle, bone, nerves and blood vessels.
      • computerized tomography scan (also called a CT or CAT scan) – a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays. They are used primarily to assess the chest and lung for metastatic tumors.
      • bone scans – a nuclear imaging method used to detect bone and metastatic tumors. It can determine if there are abnormalities in other bones. This test does not distinguish between tumor,infection or fractures.
    • Complete blood count (CBC) – a measurement of size, number, and maturity of different blood cells in a specific volume of blood
    • Blood tests (including blood chemistries)
    • Biopsy of the tumor – a small tissue sample of the tumor is surgically removed. The appearance of the tumor under a microscope establishes the diagnosis and distinguishes a fibrosarcoma from other sarcomas. Biopsy also helps doctors determine how aggressive the tumor is.

    How is fibrosarcoma staged?

    Once fibrosarcoma has been diagnosed, the tumor is staged. This process indicates how far the tumor has spread from its original location. The stage of a tumor 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, or metastatic, meaning it has spread to lungs, bones other than the bone that the tumor originated in, or to other organs or structures of the body. Tumors found during infancy are usually not metastatic.

    What are fibrosarcoma treatment options?

    Specific fibrosarcoma treatment options will be determined by your child's physician based on:

    • your child's age, overall health, and medical history
    • extent of the disease
    • your child's tolerance for specific medications, procedures, or therapies
    • how your child's physician expects the disease may progress
    • your opinion or preference

    Fibrosarcoma treatment for children may include:

    • Surgery: Surgery is often a first step, allowing doctors to form a complete diagnosis of the tumor type and providing information on the stage of the disease. If your child’s leg or arm is affected, your child may receive:
      • limb-salvage surgery, to help preserve the limb by removing the tumor and wide margins of healthy tissue surrounding the tumor
      • rotationplasty, a partial amputation that preserves a cancer-free lower leg, attaches it to the thighbone and uses the ankle as a knee joint
      • amputation surgery, which in rare cases may be necessary (for example, if it involves the nerves and blood vessels)
      • Head or neck surgery, if the fibrosarcoma is in the head or neck; this involves surgical removal of the tumor and bone/skin grafts. 

      The type of surgery will depend on the size and location of the tumor, and whether the cancer has spread. At Dana-Farber/Boston Children's, these procedures are performed in the hospital's new state-of-the-art pediatric MR-OR. It allows specialists to take an MRI scan immediately following surgery to ensure the tumor is completely removed and decreases the need for additional surgery.

    • Radiation therapy: This treatment can help stop the growth of abnormal cells 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: These medicines can help stop the growth of abnormal cells throughout the body. Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill or slow the growth of targeted cells. Different groups of chemotherapy drugs work in different ways, and can be taken in a variety of ways including orally or intravenously.
    • Rehabilitation: Rehabilitation includes physical and occupational therapy and psychosocial adapting.
    • Supportive care: Supportive care refers to any type of treatment to prevent or treat infections, side effects of treatments, and complications, and to keep your child comfortable during treatment.

    What is the long-term outlook for children with fibrosarcoma?

    Prognosis for fibrosarcoma greatly depends on:

    • the extent of the disease
    • the size and location of the tumor
    • presence or absence of metastasis
    • the tumor's response to therapy
    • the age and overall health of the child
    • your child's tolerance of specific medications, procedures, or therapies
    • new developments in treatment
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