Chondrosarcoma

  • What is chondrosarcoma?

    Chondrosarcoma is a type of malignant tumor that mimics the cartilage that coats the ends of bones where a joint is formed. Cartilage is the flexible connective tissue found in the joints between bones — around the knee for example — and also found in the rib cage, elbow, knee, ankle, bronchial tubes and spinal discs. It is not as hard as bone, instead it serves as a flexible "glue" to join one bone to another.

    Chondrosarcoma occurs mostly in adults. It is extremely rare in young children and adolescents (ages 13-19). Chondrosarcoma is most often found in the leg bone, upper arm bone, shoulder, ribs and pelvis. The malignant cancer cells can be found inside a bone or on the surface of a bone.

    Some chondrosarcomas grow very fast, while others are slow to grow and spread.

  • Causes

    Chondrosarcoma occurs when abnormal cartilage cells divide and grow. Three conditions have been connected to a higher risk of developing chondrosarcoma:

    • Ollier’s disease, when a person has many endochondromas — benign (noncancerous) tumors made up of cartilage
    • Maffucci’s syndrome, a disease where the person forms many endochondromas sporadically due to hemagiomas (blood birth-marks)
    • Bone growths called exostoses and multiple hereditary exostoses, where new bone forms on the surface of bone
  • Symptoms

    Symptoms of a chondrosarcoma depend on a variety of factors including the type of tumor, location of the tumor, and your child’s age and general health.

    Indications of a chondrosarcoma may include:

    • Pain, stiffness or tenderness at the site of the tumor
    • Swelling or mass around the affected bone
    • Enlargement of an exostosis (bone growth)
    • Limping or limited movement in the affected limb
  • Diagnosis

    Symptoms of a malignant musculoskeletal tumor can be difficult to identify in children, so regular check-ups are extremely important. Prompt diagnosis and identification of cancer as early as possible is crucial to overall treatment.

    Diagnostic evaluation begins with a thorough medical history and physical examination of your child. A detailed neurological assessment may also be performed. This involves a series of questions and tests to check the brain, spinal cord and nerve function.

    At The Children’s Hospital of Philadelphia, clinical experts use a variety of diagnostic tests to diagnose tumors, including:

    • X-rays, which produce images of bones.
    • Magnetic resonance imaging (MRI), which uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body.
    • Computed tomography (CT) scan, which uses a combination of X-rays and computer technology to produce cross-sectional images ("slices") of the body.
    • Radioisotope bone scan, which can help locate areas of abnormal growth.
    • Biopsy, which is a surgical procedure to take a sample of the growth, may be necessary to confirm any findings.
    • Spinal tap, also known as a lumbar puncture, may be performed while your child is under anesthesia to get a sample of your child’s spinal fluid to see if the tumor has spread.
    • EOS imaging, an imaging technology that creates 3-dimensional models from two planar images. Unlike a CT scan, EOS images are taken while the child is in an upright or standing position, enabling improved diagnosis due to weight-bearing positioning.
  • Treatment

    At The Children’s Hospital of Philadelphia, we practice collaborative, family-centered care. A team of expert clinicians — including leading orthopedic and oncology physicians and surgeons, advanced practice nurses and physician assistants, pediatric nurses, physical and occupational therapists, psychologists and other specialists — will partner with you in the care of your child.

    Unlike benign tumors that may only require “watchful waiting” to see if pain or dysfunction develops, malignant tumors such as chondrosarcoma require more active treatment.

    For slow-growing tumors, surgical removal of the tumor is typically the recommended treatment. For faster growing tumors, surgery and other treatments will be recommended.

    Surgery 

    Surgical treatment for chondrosarcoma may be used alone or in conjunction with chemotherapy, radiation therapy or proton therapy. 

    Surgeries performed are typically:

    • Wide resection with margins
    • Resection and reconstruction

    In a wide resection with margins, the tumor is surgically removed, as well as some of the healthy tissue that surrounded the tumor — the margins of the tumor. In some cases, the child will need reconstructive surgery after the tumor has been cut out.

    Reconstruction can include:

    • Endoprosthetics, which can be expanded as the child grows
    • Free vascularized fibular grafting, which includes moving one bone from  the lower leg to replace a diseased thigh bone
    • Amputation of a limb 
    • Other 

    Though surgery and other treatments can be effective treatments for chondrosarcoma, a cancer diagnosis and its treatments can be stressful for children and families. At CHOP, we offer a wealth of resources about how to prepare your child for surgery and what to expect during surgery.

    Additionally, we employ numerous best practices before, during and after surgery to decrease the risk of infection and increase positive outcomes. For more details about safety protocols at The Children's Hospital of Philadelphia, see safety in surgery.  

    Chemotherapy

    Chemotherapy refers to medicines that help fight cancer. They are given by mouth, in the vein, in the muscle or under the skin. Intrathecal chemotherapy is chemotherapy that is injected into the spinal fluid to prevent or treat a malignant tumor in the brain and spinal cord.

    Radiation therapy

    Radiation therapy uses high-energy waves such as X-rays to kill or shrink cancer cells. It is sometimes used to treat cancer in the central nervous system or other places such as the eye or the testes.

    Proton therapy

    Proton therapy is an innovative form of radiation treatment that allows for more precise radiation doses delivered to cancerous tumors. Proton therapy’s greatest benefit is that it is less damaging to the surrounding healthy tissue because it delivers most of its energy to a very narrow field at the location of the tumor. Learn more about proton therapy.

  • Follow-up care for chondrosarcoma

    Most children are able to go home shortly after surgery. They may require pain medications until the surgical site heals or ongoing physical therapy, but most are encouraged to resume their regular daily activities, like school, social events and play.

    Your child will see the orthopedic surgeon about one to two weeks after surgery, then again at three and six months post-surgery. Annual monitoring by trained clinicians is strongly encouraged to monitor for possible recurrence of the growth.

    Malignant tumors can reoccur — even after being successfully treated — so it is important for your child to see a physician regularly, especially if any symptoms reoccur.

    During follow-up visits, X-rays and other diagnostic testing of the tumor site are recommended to closely monitor your child’s health and ensure there are no side effects from surgery, chemotherapy or proton therapy.

    Follow-up care and ongoing support and services are available at our Main Campus and throughout our CHOP Care Network. Our team is committed to partnering with parents and referring physicians to provide the most current, comprehensive and specialized care possible for your child.

  • Long-term outcomes for chondrosarcoma

    Long-term outcomes for children with chondrosarcoma are varied and depend on the size and location of the tumor, age and overall health of your child, and how your child responded to treatment.

    With ongoing research and advances in treatment options, outcomes for children with chondrosarcoma will continue to improve. 

Reviewed by John P. Dormans, MD, FACS on May 20, 2014