Osteosarcoma

  • What is osteosarcoma?

    Osteosarcoma is the most common bone tumor among children, adolescents and young adults. This malignant musculoskeletal tumor affects approximately 400 children younger than age 20 every year in the United States.

    Osteosarcoma occurs most often in children and young adults between age 10 and 20, who are undergoing a growth spurt. It occurs more frequently in boys than in girls.

    Osteosarcoma is a malignant form of cancer, which means it tends to spread to other organs or tissues in the body, often starting with the lungs.

  • Causes

    The cause of osteosarcoma is unknown, but genetics may play an important role. Unlike some inherited childhood cancers, there is a very low risk of a child with osteosarcoma to have other family members with the same disease.

  • Signs and symptoms

    Symptoms of osteosarcoma depend on the size and location of the tumor, as well as your child’s age and general health.

    Indications of an osteosarcoma may include:

    • Chronic pain, either localized at the site of the tumor or radiating throughout part of the body
    • Decreased motor skills, including difficulty walking or limping
    • Weak bones, which may lead to a fracture
    • Fatigue
    • Fever
    • Weight loss
    • Anemia

    Osteosarcomas located in the spinal cord may cause diffuse symptoms that can affect any part of the body.

  • Testing and diagnosis

    Symptoms of osteosarcoma can be challenging 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 your child's 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.
    • Positron emission tomography (PET) scan, which uses radioactive sugar injected into a vein and a scanner to make detailed, computerized pictures of areas of the body.
    • Blood tests, which can help determine drug usage and effectiveness, biochemical diseases and organ function.
    • Biopsy, which is a surgical procedure to take a sample of the growth, may be necessary to confirm any findings.
    • 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.

    In addition to diagnosing the specific type of cancer your child may have, these tests will also help determine the size, location and stage of the cancer. All of this information is crucial in determining the best treatment options for your child.

  • Treatments

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

    Chemotherapy

    Treatment for osteosarcoma, a particularly aggressive form of cancer, always involves chemotherapy before and after surgery to kill the main tumor and kill cancer cells that may have already spread, but have not yet been detected. Chemotherapy refers to medicines that help fight cancer. They may be given to your child orally by mouth, or injected in a vein, muscle or under the skin.

    Surgery

    Surgical treatment for osteosarcoma may be used 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.

    Reconstructive surgery

    Reconstruction can include:

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

    Limb-sparing surgery 

    Surgical orthopedic and oncology specialists at CHOP are equipped to perform complex, limb-sparing surgeries for children with cancers like osteosarcoma. CHOP surgeons are constantly pushing the envelope to improve upon existing surgical techniques, and improve outcomes for patients with even the most difficult-to-treat tumors. Learn more about limb-sparing surgery in the video below.

    Surgical safety

    Though surgery for soft tissue tumors is highly effective, we understand that any surgery can be a stressful experience 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. Download CHOP's Having Surgery booklet (PDF).

    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.

    Radiation therapy

    Depending on your child’s individual situation, radiation therapy may an option to treat osteosarcoma. 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. 

  • Follow-up care

    If surgery was performed, your child will be examined by the 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 and manage any side effects of treatment.

    Osteosarcoma 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.

  • Outlook

    The Children's Hospital of Philadelphia has an excellent track record of treating children diagnosed with osteosarcoma.

    As with any cancer, prognosis and long-term survival can vary greatly from child to child, however. Prompt medical attention and aggressive therapy are important for the best prognosis.

Reviewed by John P. Dormans, MD, FACS, Jason L. Katz, PA-C, MHS, ATC-R on May 20, 2014