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Chondrosarcoma

  • Overview

    Chondrosarcoma is a type of cancer that resembles the cartilage that coats the ends of bones and forms joints.

    • Chondrosarcoma occurs primarily in adults, are rarely encountered during the adolescent years and almost never affect young children.
    • Chondrosarcoma most commonly occurs in cartilage found in the femur, humerus, shoulder, ribs and pelvis.
    • It can occur inside the bone or on the surface of the bone.
    • It can be a rapidly growing invasive tumor or it can develop slowly, causing less severe symptoms and sometimes never spreading.

    Cancer research at Boston Children's Hospital

    Dana-Farber/Children's Hospital Cancer Care researchers are conducting numerous research studies that will help clinicians better understand and treat all kinds of tumors. Some types of treatment currently being studied include:

    • Angiogenesis inhibitors - substances that may be able to prevent the growth of tumors by blocking the formation of new blood vessels that feed the tumors
    • Biological therapies - a wide range of substances that may use the body's own immune system to fight cancer or lessen harmful side effects of some treatments

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    Boston Children's Hospital
    300 Longwood Avenue
    Fegan 2
    Boston MA 02115

     617-355-6021



  • In-Depth

    What causes chondrosarcoma?

    Chondrosarcoma occurs when a single abnormal cartilage cell divides out of control.

    Scientists are not certain what causes this to occur, although several factors may put a person at risk:

    • Ollier's disease
    • Maffuci's syndrome
    • Exostoses (bone growths)

    Chondrosarcoma is extremely rare in children. There is some relationship between chondrosarcoma and repeated trauma to the affected area, but it is unclear whether injury puts a person at risk for this type of cancer, or the cancer itself makes the area weaker, thus making injury more likely.

    What are the symptoms of chondrosarcoma?

    Each child may experience symptoms differently. Some symptoms that your child may have include the following:

    • pain (sharp or dull) at the site of the tumor
    • swelling and/or redness at the site of the tumor
    • enlargement or growing of a pre-existing exostosis (bone growth)
    • increased pain with activity or lifting
    • limping
    • decreased movement of the affected limb
    • urinary frequency or obstruction (in pelvic tumors)
  • Tests

    How is a chondrosarcoma diagnosed?

    Diagnostic procedures for chondrosarcoma are used to determine the exact type of tumor your child has and whether the tumor has spread.

    This may include:

    • Physical exam and neurologic function tests including reflexes, muscle strength, eye and mouth movement, coordination and alertness
    • Magnetic resonance imaging (MRI) to produce detailed images of organs and structures within the body and/or spine
    • Magnetic resonance spectroscopy (MRS) which is a test done along with MRI at specialized facilities that can detect the presence of particular organic compounds produced by the body's metabolism within sample tissue
      • This helps us identify tissue as either normal or tumor, and may be able to distinguish between glial tumors and tumors of neuronal origin.
    • Computerized tomography scan (also called a CT or CAT scan) to capture a detailed view of the body
    • Biopsy or tissue sample from the tumor to provide definitive information about the type of tumor
      • This is collected during surgery.
    • Lumbar puncture (spinal tap) to remove a small sample of cerebrospinal fluid and determine if any tumor cells have started to spread.
    • In young children, this procedure is safely performed under sedation, and is less difficult and less painful than placing an intravenous (IV) catheter.

    Staging

    Once chondrosarcoma 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 chondrosarcoma?

    Treatment for a low-grade chondrosarcoma involves an operation to remove the tumor. This kind of surgery alone is usually sufficient to cure these less-invasive tumors.

    A rapidly growing, high-grade chondrosarcoma may include surgery combined with other forms of therapy, which are discussed below.

    Surgery

    Surgery for chondrosarcoma 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.

    Children 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, children who undergo limb-sparing surgery may eventually have to have the limb amputated because of a severe complication or tumor recurrence.

    Amputation

    If your child's orthopedic surgeon 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.

    Your child 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

    This therapy is rarely used in chondrosarcoma except for extremely aggressive forms of the disease.

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

    • Different groups of chemotherapy drugs work in different ways to fight cancer cells and shrink tumors.
    • Often, a combination of chemotherapy drugs is used.
    • Certain chemotherapy drugs may be given in a specific order depending on the type of cancer it is being used to treat.

    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 needle)

    Rehabilitation

    Rehabilitation includes physical and occupational therapy along with psychosocial counseling.

    Supportive care

    Supportive care is 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 chondrosarcoma?

    Prognosis for chondrosarcoma 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 chondrosarcomas?
    Children treated for chondrosarcomas 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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