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300 Longwood Avenue
Boston, MA 02115
(617) 355-6000
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My Child Has:
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Osteosarcoma
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Also called osteogenic sarcoma, osteosarcoma is one of the most common types of bone cancer in children.
The disease usually occurs in the long bones, such as the arms (humerus), legs (femur/tibia), and pelvis. It rarely occurs in the jaw and fingers, but often occurs at the ends of these bones near growth plates. Osteosarcoma affects adolescents and young adults.
Osteogenic sarcoma takes many forms. While the classic form of the disease originates in the marrow cavity of the bone, some variations originate in cells forming just outside of or at the surface of the bone. The most common of these variants is parosteal osteosarcoma, which originates in a layer of soft tissue adjacent to the bone. This tumor tends to grow slow, eventually invading the underlying bone. It tends to occur in people between the ages of 20 and 40.
The most common site for this type of tumor is the posterior side of the distal (lower end) femur.
Another type is called periosteal osteosarcoma. This type originates in the fibrous sheath that covers the bone -- the periosteum, which contains the blood vessels and nerves that provide nourishment and sensation to the bone. Periosteal osteosarcoma, which is more aggressive than parosteal osteosarcoma, is frequently located in the proximal (upper end) of the tibia.
Osteosarcoma cancer cells can also spread (metastasize) to other areas of the body. Most commonly, these cells spread to the lungs. However, bone, and much less commonly, kidneys, the adrenal gland, the brain, and the heart can also be sites of metastasis.
As you read further below, you will find general information about osteosarcoma. If you would like to view summary information about cancer first, see the cancer overview.
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It has been suggested that repeated trauma to an area may be a risk factor for developing this type of cancer. It is uncertain whether trauma is a cause or effect of the disease. Cancer lesions in the bone can make that area of the bone weaker, thus, making fracture more likely. However, repeated injuries to a certain area of the bone may lead to an increased production of osteoid tissue to repair the damaged area. The rapid production of osteoid tissue may lead to the malignancy. It is thought, most often, that injury simply brings the condition to attention and has no causal relationship.
Genetics may play an important role in developing osteosarcoma. Children and adults with other hereditary abnormalities, including exostoses (bony growths), retinoblastoma, Ollier's disease, osteogenesis imperfecta, polyostotic fibrous dysplasia, and Paget's disease, have an increased risk for developing osteosarcoma. This form of cancer has also been linked to exposure to ionizing irradiation associated with radiation therapy for other types of cancer (i.e., Hodgkin's disease and non-Hodgkin's lymphoma).
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The following are the most common symptoms of osteosarcoma. However, each child may experience symptoms differently. Symptoms may include, but are not limited to, the following:
- 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
The symptoms may have been present over a short period of time or may have been occurring for six months or more. Often, an injury brings a child into a medical facility, where an x-ray may indicate suspicious bone lesions. Usually, the child does not appear to be ill.
The symptoms of osteosarcoma may resemble other conditions or medical problems. Always consult your child's physician for a diagnosis.
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In addition to a complete medical history and physical examination of your child, diagnostic procedures for osteosarcoma may include:
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- multiple imaging studies of the tumor and sites of possible metastasis, such as:
- X-rays - a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto filmX-rays are very useful in the diagnosis of bone tumors and frequently x-rays allow the physician to distinguish between benign and malignant tumors. An x-ray is usually the first diagnostic study and it often gives the doctor information regarding the need for further testing.
- bone scans - a nuclear imaging method to detect bone and metastatic tumors. It can determine if there are abnormalities in other bones. This test does not distinguish between tumor, infection or fractures.
- magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. 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 diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. 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. They are used primarily to assess the chest and lung for metastatic tumors.
- complete blood count (CBC) - a measurement of size, number, and maturity of different blood cells in a specific volume of blood
- blood tests (including blood chemistries)
- biopsy of the tumor - a small tissue sample of the tumor is surgically removed. The tumor's appearance under a microscope best distinguishes it from other bone tumors and establishes the diagnosis.
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Once osteosarcoma has been diagnosed, the tumor is staged. This process indicates how far the tumor has spread from its original location. The stage of a tumor suggests which form of treatment is most appropriate, and gives some indication of the prognosis of the condition.
An osteosarcoma may be localized, meaning it has not spread beyond the bone where it arose or beyond nearby tissues, or metastatic, meaning it has spread to lungs, bone other than bone that the tumor originated in, or to other organs or structures of the body.
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Specific treatment for osteosarcoma will be determined by your child's physician based on:
- your child's age, overall health, and medical history
- extent and location of the disease
- your child's tolerance for specific medications, procedures, or therapies
- how your child's physician expects the disease may progress
- your opinion or preference
Treatment may include, but is not limited to, one or more of the following:
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Surgery for osteosarcoma involves biopsy, surgical removal of the tumor, bone 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.
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- Limb-salvage surgery: It is sometime necessary to remove all or part of a limb. In most cases, however, limb-salvage surgery is used to avoid amputation. This however is considered only if the orthopedic surgeon determines that it is possible that the tumor, and wide margins of healthy tissue surrounding the tumor, can be removed.
Through limb-salvage 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 the reconstruction which can become loose or break. Patients who have undergone limb-salvage surgery need intensive rehabilitation. It may take as long as a year for a patient to regain full use of a limb following limb-salvage surgery. Rarely, some patients who have this operation may eventually have to undergo amputation. Chemotherapy is usually given before surgery to kill the tumor, and after surgery to treat remaining cancer cells that my have spread elsewhere in the body. For very young children a special metallic prosthesis that can be lengthened as the child grows is sometimes employed for limb salvage reconstruction.
- Amputation - In some cases, if your child's orthopedic surgeon determines that the tumor cannot be removed because, for example, it involves the nerves and blood vessels, amputation is the only option.
During the operation, doctors ensure that muscles and skin form a cuff around the amputated bone. A cast may be applied in the operating room which permits a temporary artificial leg (prosthesis) to be applied during the first few post-operative days for walking. Crutches are used for several weeks. As the swelling decreases (10 to 14 days) the patient is fitted for a plastic, temporary socket and prosthesis, which is used for 2 to 4 months until the stump is healed sufficiently to accept a permanent artificial leg. 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 Rotationplasty, a variation of amputation for tumors in the thigh (femur). This operation preserves the lower leg and uses the ankle as a knee joint. In this procedure, the patient functions more like a below knee amputation rather than an above knee amputation. It is especially useful in very young children where limb length would be an issue.
- rotationplasty - This operation preserves the lower leg and uses the ankle as a knee joint. In this procedure, the patient functions more like a below knee amputee rather than an above knee amputee. It is especially useful in very young children where limb length would be an issue.
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Chemotherapy is a drug treatment that works by interfering with the cancer cell's ability to grow or reproduce. Different groups of drugs work in different ways to fight cancer cells and shrink tumors. Chemotherapy may be used alone for some types of cancer or in conjunction with other therapy such as radiation or surgery. Often, a combination of chemotherapy drugs is used to fight a specific cancer. 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 do not 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.
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Radiation uses high-energy rays from a specialized machine to damage or kill cancer cells and shrink tumors. This modality is seldom used for osteosarcoma.
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Rehabilitation includes physical and occupational therapy and psychosocial adapting
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Supportive care refers to any type of treatment to prevent and treat infections, side effects of treatments, and complications, and to keep your child comfortable during treatment.
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A schedule of follow-up care is 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.
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Prognosis for osteosarcoma 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 percent of tumor cells killed following chemotherapy)
- the age and overall health of the child
- your child's tolerance of specific medications, procedures, or therapies
- new developments in treatment
As with any cancer, prognosis and long-term survival can vary greatly from child to child. Every child is unique and treatment and prognosis is structured around the child's needs. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a child diagnosed with osteosarcoma. Side effects of radiation and chemotherapy, as well as second malignancies, can occur in survivors of osteosarcoma. New methods are continually being discovered to improve treatment and to decrease side effects.
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Children's Hospital and Dana-Farber Cancer Institute are conducting numerous research studies that will help clinicians 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. In addition, a study of high-doses of a medication called doxorubicin continues for patients with high-risk osteosarcoma.
Other 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 be able to involve the body's own immune system to fight cancer or lessen harmful side effects of some treatments
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The information on this website should not be taken as medical advice,
which can only be given to you by your personal health care professional. |
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Copyright © Children's Hospital Boston. All rights reserved. |
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