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

Symptoms of osteosarcoma may resemble other, more common, conditions or medical problems. If you suspect that your child has osteosarcoma, always consult your child's physician for a diagnosis.

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 lymphoma) increases the risk of osteosarcomas.

How is osteosarcoma diagnosed?

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-ray
• magnetic resonance imaging (MRI)
• computerized tomography scan (also called a CT or CAT scan)
• biopsy or tissue sample
• bone scan
• blood tests
• heart evaluation
• hearing test
• evaluation of kidney function prior to the start of chemotherapy

What are the treatment options for osteosarcoma?

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.

Surgery for osteosarcoma

Depending on the size and location of the tumor and whether the tumor has spread, your child may receive one or more of several surgical treatments. Limb-salvage surgery helps preserve the limb by removing the tumor and wide margins of healthy tissue surrounding the tumor. The goal of limb-salvage surgery is to preserve limb function, as well as the pre-surgical appearance of the limb. However, 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, skateboarding, or bike riding.

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. One of the major benefits of rotationplasty compared to other surgical options is that it allows the child to maintain a very active lifestyle, including high-impact sports.

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 and rotationplasty are complex procedures that require specialized expertise. Our bone tumor surgeons are among the few orthopedic surgeons in the nation trained in pediatric tumor surgery and who have experience in these procedures. At Dana-Farber/Boston Children's, we also have physical therapists and prostheticians who specialize in helping children who undergo these procedures.

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 and 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 — in some cases, prevent — these symptoms.

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. Boston Orthotics & Prosthetics of 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 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.

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

Dana-Farber/Boston Children’s 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.

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