KidsMD Health Topics

Fibrosarcoma

  • A fibrosarcoma is a cancerous tumor that originates in the connective tissue found at the ends of bones of the arm or legs, and then 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
    • other fibrous tissue

    Fibrosarcoma most commonly affects either a lower leg or arm. There are generally two forms of this disease:

    • Infantile or congenital fibrosarcoma - This type of tumor is the most common soft tissue sarcoma found in children under one year of age. It's usually slow-growing, and tends to be more benign than fibrosarcoma in older children.
    • 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.

    What causes fibrosarcoma?

    The exact cause of fibrosarcoma and other soft tissue tumors is not entirely understood. However, studies have indicated that genetic alterations may be involved.

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  • In-Depth

    What are the symptoms of fibrosarcoma?

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

    Your child's symptoms may include:

    • a painless or tender mass in her extremity or trunk
    • pain or soreness caused by suppressed nerves and muscles
  • How is fibrosarcoma diagnosed?

    Your child's doctor may recommend the some or all of the following tests:

    • X-ray - X-rays are very useful in the diagnosis of bone tumors. They are the first diagnostic study, and they often give your doctor information regarding the need for further testing.
    • Magnetic resonance imaging (MRI) - This test outlines the extent of the tumor within the bone and joint and the relationship of the tumor to the muscles, nerves and blood vessels.
    • Computerized tomography scan (also called a CT or CAT scan) - 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.
    • Bone scans - A nuclear imaging method used to detect bone and metastatic tumors. Bone scans 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.
    • Biopsy of the tumor - A small tissue sample of the tumor is surgically removed and examined under a microscope. This procedure enables your doctor to establish the diagnosis and distinguishes fibrosarcoma from other sarcomas. Biopsy also helps your doctor determine how aggressive the tumor is.

    Staging

    Once fibrosarcoma has been diagnosed, the tumor is staged. This process shows how far the tumor has spread from its original location. The stage of the tumor and grade of the tumor (how aggressive the tumor is) suggests which form of treatment is most appropriate, and gives us an idea of the prognosis.

  • What's the treatment for fibrosarcoma?

    Congenital fibrosarcoma

    • Surgery may be all that is needed for an infant with this condition, and limb-sparing surgery is often opted for, rather than amputation, since a local recurrence of congenital fibrosarcoma is not believed to worsen a child's prognosis.
    • Radiation (explained further below) is not generally used for tumors in the extremities because it may interfere with proper bone growth.
    • Chemotherapy (also discussed below), which is very effective on this type of tumor, may be used in infants if appropriate resection of the tumor with wide surrounding margins of healthy tissue is not possible.

    Adult-form fibrosarcoma

    Older children with this condition are more likely to undergo a combination or surgery, chemotherapy and/or radiation treatment.

    Surgery

    Surgery for fibrosarcoma involves the biopsy, surgical removal of the tumor, bone/skin grafts, limb salvage procedures, amputation and/or reconstruction, all performed by a surgeon. The type of surgery will depend on the size and location of the tumor, and whether the cancer has spread.

    Limb-sparing surgery

    It is sometimes necessary to remove all or part of a limb. In most cases, however, limb-sparing surgery is used to avoid amputation.

    • Through limb-sparing surgery, all of the bone and cartilage involved with the tumor, including some degree of muscle surrounding it, is removed, while nearby tendons, nerves and vessels are saved.
    • The bone that is removed is replaced with a bone graft or with a metal prosthesis.
    • Subsequent surgery may be needed to repair or replace rods, which can become loose or break.
    • Patients who have undergone limb-sparing surgery need intensive rehabilitation. It may take as long as a year for your child to regain full use of a limb following limb-sparing surgery.
    • Rarely, patients who undergo limb-sparing surgery may eventually have to have the limb amputated because of a severe complication or tumor

    Amputation

    If your child's team determines that the tumor cannot be removed because it involves important nerves and blood vessels, amputation is the only surgical option.

    During the operation, doctors ensure that muscles and skin form a cuff around the amputated bone. As the swelling decreases, (10 to 14 days), your child will be fitted for a plastic, temporary socket and prosthesis, which is used for two to four months until the stump is healed sufficiently to accept a permanent artificial limb.

    The advantages of an amputation are that it is a simple operation with minimal chances of surgical complication and it definitively removes the local tumor. The functional outcome is good with the modern prostheses available today and with "immediate-fit" prostheses applied in the operating room.

    Although your child will probably have a limp with above-the-knee amputations, the procedure is functional and stable.

    She will be able to walk, climb stairs, swim (with the prosthesis on or off) and participate in many sports such as skiing, basketball, baseball and tennis, although running will be limited.

    Chemotherapy

    Chemotherapy is a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce.

    While chemotherapy can be quite effective in treating certain cancers, the agents don't differentiate normal healthy cells from cancer cells.

    Because of this, there can be many adverse side effects during treatment. Being able to anticipate these side effects can help the care team, parents, and child prepare, and, in some cases, prevent these symptoms from occurring, if possible.

    Chemotherapy is systemic treatment, meaning it is introduced to the bloodstream and travels throughout the body to kill cancer cells. Chemotherapy can be given:

    • as a pill to swallow
    • as an injection into the muscle or fat tissue
    • intravenously (directly to the bloodstream)
    • intrathecally (directly into the spinal column with a

    Rehabilitation

    Rehabilitation includes physical and occupational therapy along with psychosocial

    Supportive care

    Supportive care s any type of treatment to prevent and treat infections, side effects of treatments, and complications, and to keep your child comfortable during treatment

    Continual follow-up care

    A schedule of follow-up care will be determined by your child's physician and other members of your care team to monitor ongoing response to treatment and possible late effects of treatment.

    What is the long-term outlook for patients 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 your child
    • your child's tolerance of specific medications, procedures, or therapies
    • new developments in treatment

    Every child is unique and treatment and prognosis is structured around your child's needs. Prompt medical attention and aggressive therapy are important for the best prognosis.

    What is the recommended long-term care for children treated for fibrosarcoma?

    Children should visit a survivorship clinic every year to:

    • manage disease complications
    • screen for early recurrence of cancer
    • manage late effects of treatment

    A typical follow-up visit may include some or all of the following:

    • a physical exam
    • laboratory testing
    • imaging scans

    Through the David B. Perini, Jr. Quality of Life Clinic at Dana-Farber Cancer Institute, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team.

    • Our childhood cancer survivorship clinic is held weekly.
    • In addition to meeting with your pediatric oncologists, your child may see one of our endocrinologists, cardiologists, neurologists, neuro-psychologists or alternative/complementary therapy specialists.

          We also offer the following services:

    • patient and family education
    • psychosocial assessment
    • genetic counseling
    • reproductive and fertility evaluation and counseling
    • opportunities to speak with other childhood cancer survivors
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