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Osteosarcoma

  • Osteosarcoma is the most common type of bone cancer in children and young adults. It usually occurs in the long bones, such as the arms or legs, though it can also occur in the pelvis and spine. In rare cases, it can be in the jaw or other locations in the body.

    How Dana-Farber/Boston Children’s approaches osteosarcoma in children

    Dana-Farber/Boston Children’s Cancer and Blood Disorders Center provides comprehensive medical and surgical care for children and adolescents with bone tumors, such as osteosarcoma. Our multidisciplinary approach through our Bone and Soft Tissue Program ensures in-depth discussion of each case and personalized treatment plans for every patient. We integrate expertise from the following specialists:

    • pediatric oncologists, oncologic orthopedists and oncologic surgeons
    • pediatric experts from every medical subspecialty, such as physical therapy and radiology, among others
    • highly skilled and experienced pediatric oncology nurses
    • child life specialists, psychologists, social workers and resource specialists who provide supportive care before, during and after treatment

    Our specialists help develop and participate in the latest national research protocols and treatments for osteosarcoma, providing the best standard of care. Our orthopedic surgeons and pediatric oncologists have in-depth experience in the care of osteosarcoma and other pediatric bone tumors.

    “Translational research is about what we can do to bring science from the bench to the bedside. We have some of the best scientists and translational researchers in the country for pediatric solid tumors working on this initiative."
    - Carlos Rodriguez-Galindo, MD

    Osteosarcoma: Reviewed by Katherine Janeway, MD
    © Dana-Farber/Boston Children’s, 2013

  • What is osteosarcoma?

    Osteosarcoma is the most common type of bone cancer among children, adolescents and young adults. It usually begins in the marrow cavity of the long bones, such as the arms and legs. It also can occur in the pelvis and spine, and in rare cases, in the jaw or other locations in the body.

    While osteosarcoma occurs most commonly in teenagers, when the rate of bone growth is the fastest, it can affect young children and adults as well. There are about 600 new cases of osteosarcoma diagnosed each year in the United States.

    Types of osteosarcoma Some types of osteosarcoma can start just outside or at the surface of the bone, instead of in the marrow cavity. These include:

    Parosteal osteosarcoma

    • originates in a layer of soft tissue next to the bone
    • tends to grow slowly, eventually invading the bone
    • tends to occur in people between the ages of 20 and 40
    • most commonly forms in the back and the lower end of the thighbone

    Periosteal osteosarcoma

    • originates in the tough outer covering that surrounds the bone and contains the blood vessels and nerves
    • more aggressive than parosteal osteosarcoma
    • usually located in the upper end of the leg bone

    Osteosarcoma cancer cells can spread (metastasize) to other areas of the body. If this happens, it most often spreads to the lungs. It also can spread to other bones, and much less commonly, to the kidneys, the adrenal gland, the brain and the heart.

    Causes of Osteosarcoma

    What causes osteosarcoma?

    In most cases, there is no clear cause for osteosarcoma. Genetics, abnormal bone metabolism or environmental exposures play an important role in some people.

    • Inherited conditions that increase the risk of osteosarcoma are hereditary retinoblastoma, Li-Fraumeni Syndrome and Rothmund-Thomson, Bloom and Werner syndromes.
    • Bone diseases associated with an increased risk of osteosarcoma include Ollier's disease, osteogenesis imperfecta, polyostotic fibrous dysplasia and Paget's disease.
    • Radiation therapy for other types of cancer (such as Hodgkin's disease and non-Hodgkin's lymphoma) increases the risk of osteosarcoma.

    Symptoms of Osteosarcoma

    What are the symptoms of osteosarcoma?

    The symptoms of osteosarcoma can vary depending on the size and location of the tumor and whether it has spread.

    Often, an injury brings your child into a medical facility, where an x-ray may indicate suspicious bone lesions. Usually, your child does not appear to be ill. While each child may experience symptoms differently, the most common include:

    • pain (sharp or dull) at the site of the tumor
    • swelling (mass) and/or redness at the site of the tumor
    • increased pain with activity or lifting
    • limping
    • decreased movement of the affected limb

    Keep in mind that the symptoms of osteosarcoma may resemble other, more common conditions or medical problems, so if you suspect that your child has osteosarcoma, always consult your child's physician for a diagnosis.

  • How does my child’s doctor know that it’s osteosarcoma?

    Diagnostic procedures for osteosarcoma are used to determine the exact type of tumor your child has and whether the tumor has spread. Your child’s doctor will perform a complete medical and physical examination. In addition, your child’s physician may order some of the following diagnostic tests for osteosarcoma:

    • x-rays to produce images of the area of pain and/or swelling
    • magnetic resonance imaging (MRI) of the area of pain and/or swelling to produce detailed images of the tumor and adjacent parts of the body
    • computerized tomography scan (also called a CT or CAT scan) of the chest and sometimes of other parts of the body to determine whether the tumor has spread
    • biopsy or tissue sample from the tumor to provide definitive information about the type of tumor; this is usually collected by a radiologist using a minimally invasive approach but can sometimes require surgery
    • bone scan to determine whether the tumor has spread
    • blood tests, heart evaluation, hearing test and evaluation of kidney function prior to the start of chemotherapy
  • Treatments for osteocarcoma may involve a combination of therapies including surgery, radiation and/or chemotherapy. In most cases, children receive chemotherapy before surgery (neoadjuvant), a surgical procedure to remove the tumor and additional chemotherapy after surgery (adjuvant). Treatment options will vary greatly, depending on your child's situation. Your child's doctor and other members of your care team will discuss the options with you in-depth. Prompt medical attention and aggressive therapy are important for the best prognosis.

    Children with osteosarcoma are treated through the Bone and Soft Tissue Program at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, an integrated pediatric hematology and oncology partnership between Dana-Farber Cancer Institute and Boston Children’s Hospital.

    Surgery for Osteosarcoma

    Depending on the size and location of the tumor and whether the tumor has spread, your child may receive one of the following surgical treatments:

    • Limb-salvage surgery helps preserve the limb by removing the tumor and wide margins of healthy tissue surrounding the tumor. This type of surgery is done whenever possible as long as the tumor can be completely removed using this method. The goal of limb-salvage surgery is to preserve as much use of the limb as possible and preserve limb function.
    • Amputation may be necessary if the tumor cannot be completely removed (for example, if it involves the nerves and blood vessels) or if limb function cannot be preserved through limb-salvage surgery. If amputation is necessary, your child may be fitted for a prosthesis following surgery.
    • Rotationplasty is a partial amputation that preserves a cancer-free lower leg, attaches it to the thighbone and uses the ankle as a knee joint—especially useful in very young children where limb length can be an issue. This operation may be performed when the tumor is very large and complete removal would be difficult.

    Surgery at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center is personalized to help your child achieve the best functional and cosmetic outcome and to allow them to remain active and healthy. Depending on the type of surgery your child receives, your child may have to avoid certain physical activities following treatment. Limb-salvage surgery can leave an arm or leg fragile and increase the risk of fracture. As a result, patients will need to avoid high-stress physical activities, such as skiing, skate-boarding or bike riding. Children who require partial or complete limb amputations will be fitted with a prosthesis and need to undergo physical therapy to adapt to using it.

    Talk to your child’s pediatric oncologist or orthopedic surgeon for more information on these procedures.

    If the osteosarcoma has spread to other parts of the body, such as the lungs, additional surgery may be required, and our oncologic surgeons will remove any metastases that may have formed.

    Chemotherapy for Osteosarcoma

    Chemotherapy is a group of drugs that interfere with the cancer cell’s ability to grow or reproduce.

    • Different groups of chemotherapy drugs work in varied 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.

    Does chemotherapy cause side effects?

    While chemotherapy can be quite effective in treating certain cancers, the drugs cannot tell the difference between normal healthy cells and cancer cells. Because of this, your child may experience adverse side effects during treatment. Understanding these side effects can help you, your child and your care team prepare for, and, in some cases, prevent these symptoms from occurring.

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

    • orally, as a pill to swallow
    • intramuscularly (or IM), as an injection into the muscle or fat tissue
    • intravenously (or IV), directly to the bloodstream
    • intrathecally, with a needle directly into the fluid surrounding the spine

    Rehabilitation

    Rehabilitation is an extremely important part of your child’s osteosarcoma care. This includes physical and occupational therapy, as well as help adapting to social situations. Your child’s physician will discuss what types of lifestyle changes may be necessary, and our experienced physical and occupational therapists will work to optimize his mobility after surgery.

    Some cases of osteosarcoma require partial or complete amputation of an arm or leg. The NOPCO (National Orthotics and Prosthetics Company) Brace Shop at Boston Children's Hospital provides each patient with an expertly made, customized prosthesis, and our physical therapists help children adjust to using them.

    Supportive care

    Supportive care is treatment to prevent and treat infections, side effects of treatments and complications. The goal is to keep your child comfortable during treatment. It also is an important part of preventing short- and long-term complications of the disease and treatment.

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

    Your child’s prognosis (chance of recovery) and treatment options depend on a number of different factors, including:

    • the extent of the disease
    • the size and location of the tumor
    • the 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

    In general, osteosarcomas in the limbs tend to be more treatable. Osteosarcomas in other locations are more difficult to treat and require more aggressive therapy. Prompt medical attention and appropriate therapy are important for the best prognosis.

    What treatments are available for relapsed or recurrent osteosarcoma?

    Unfortunately, about 40 percent of children treated for osteosarcoma will experience relapse, or a return of the disease.

    Our pediatric oncologists and surgeons are experienced in treating relapsed osteosarcoma. For children with relapsed osteosarcoma, we offer the highest standard of care including additional surgery and chemotherapy. We also provide access to newly developed treatments through phase I and phase II clinical trials at Dana-Farber/Boston Children’s and the Children’s Oncology Group.

  • Childhood cancer was once considered to always be fatal, but today, the majority of pediatric cancer patients can expect to be long-term survivors.

    Survivorship comes with numerous complex issues: the long-term effects of treatment and the risk of second cancers, as well as social and psychological concerns. Children treated for osteosarcoma may require long-term physical and occupational therapy and in some cases may need a brace or prosthesis to help them remain mobile and active.

    In addition, children treated for osteosarcoma 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

    Since 1993, physicians, nurses, researchers and psychologists in the David B. Perini, Jr. Quality of Life Clinic at Dana-Farber Cancer Institute have helped thousands of survivors of pediatric cancers, treated at the institute and at other hospitals, to manage these long-term consequences.

    Here are some services that you will encounter at the Perini Clinic:

    • weekly childhood cancer survivorship clinic
    • meetings with your pediatric oncologists, endocrinologists, cardiologists, neurologists, neuropsychologists or alternative/complementary therapy specialists
    • patient and family education
    • psychosocial assessment
    • genetic counseling
    • reproductive and fertility evaluation and counseling
    • opportunities to speak with other childhood cancer survivors

    Contact Us

    To make an appointment at the Perini Clinic, please contact the clinic's facilitator at (617) 632-5124 or email Perini_Clinic@dfci.harvard.edu

  • Dana-Farber/Boston Children’s Cancer and Blood Disorders Center was one of the first centers in the United States to use adjuvant chemotherapy and perform limb salvage surgery for patients with osteosarcoma.

    Apart from our very active basic research program on osteosarcoma and phase I and phase II clinical trials at Dana-Farber/Boston Children’s, we also have available all Children’s Oncology Group clinical trials for newly diagnosed, non-metastatic and metastatic osteosarcoma.

    Here is some of the investigations that our researchers are currently working on:

    • We are studying angiogenesis inhibitors, substances that may be able to prevent the growth of tumors.
    • We are investigating biological therapies, a wide range of substances that may be able to involve the body's own immune system to fight cancer or lessen harmful side effects of some treatments.
    • We are working to improve supportive care and reduce the side effects of chemotherapy to increase the effectiveness of treatment and prevent short- and long-term complications.

    We also are conducting numerous research studies to help better understand and treat osteosarcoma. These researchers are directing and participating in national and international clinical trials to develop new treatments for patients with newly diagnosed osteosarcoma or relapsed disease, using surgery, radiation therapy and chemotherapy to more effectively overcome cancer.

    Clinical trials

    For many children with rare or hard-to-treat conditions, clinical trials provide new options.

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