Osteosarcoma

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    Osteosarcoma is a cancer of the bone that usually affects the large bones of the arm or leg. It occurs most commonly in young people and affects more males than females. Learn about osteosarcoma and find information on how we support and care for children and teens with osteosarcoma before, during, and after treatment.

The Solid Tumor Center at Dana-Farber/Boston Children's Cancer and Blood Disorders Center treats children and teens with a variety of solid malignancies, including bone and soft tissue tumors, liver and kidney tumors, neuroblastomas, retinoblastomas and rare tumors. Our doctors provide unparalleled expertise in the diagnosis, treatment and management of these diseases.

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Information for: Patients | Healthcare Professionals

Osteosarcoma

Overview

A diagnosis of osteosarcoma, a rare type of bone cancer that occurs most often in teenagers, is extremely difficult for any parent. Osteosarcoma is a type of bone cancer that usually occurs in the long bones, such as the arms or legs. It can also occur in the pelvis and spine. In rare cases, it can be in the jaw or other locations in the body.

  • It’s the most common type of bone cancer in children and young adults.
  • There are about 600 cases of osteosarcoma in the United States per year.
  • Osteosarcoma occurs most commonly in teenagers, when the rate of bone growth is the fastest. However, it can affect young children and adults as well.
  • It can spread to other areas of the body including lungs, bone, and much less commonly, kidneys, the adrenal gland, the brain, and the heart.
  • The cause is unknown, but genetics can play a role.
  • Treatment most often involves surgery and chemotherapy.

How Dana-Farber/Boston Children's Cancer and Blood Disorders Center approaches osteosarcoma

Our multidisciplinary approach to care 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 orthopedics, ophthalmology, 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

We provide the option of participating in the latest national research protocols or treatment with 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

In depth

You probably have a lot of questions: How dangerous is osteosarcoma? What is the very best treatment? What do we do next?

We’ve tried to provide some answers to those questions here, and our expert specialists can explain your child’s condition fully when we meet with you.

What is osteosarcoma?

Osteosarcoma is a type of bone cancer that usually occurs in the long bones, such as the arms and legs. It can also occur in the pelvis and spine. In rare cases, it can occur in the jaw or other locations in the body.

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

Types of osteosarcoma

Most commonly, the disease begins in the marrow cavity of the bone. However, some types of osteosarcoma can start just outside of or at the surface of the bone. 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
     
What causes osteosarcoma?

As a parent, you undoubtedly want to know what may have caused your child’s tumor. 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.
  • The environmental exposure known to increase the risk of osteosarcoma is exposure to ionizing irradiation associated with radiation therapy for other types of cancer (such as Hodgkin's disease and non-Hodgkin's lymphoma).

It’s important to understand that osteosarcoma most often occurs with no known cause. There’s nothing that you could have done or avoided doing that would have prevented the tumor from developing.

Is osteosarcoma common?

Osteosarcoma is the most common type of bone cancer among children, adolescents and young adults.

  • There are about 600 new cases of osteosarcoma diagnosed each year in the United States.
  • Osteosarcoma occurs most commonly in teenagers, when the rate of bone growth is the fastest. However, it can affect younger children and adults as well.
What are the symptoms of osteosarcoma?

Each child may experience symptoms differently. 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 symptoms may vary child-to-child, 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 may have osteosarcoma, always consult your child's physician for a diagnosis.

FAQ

Q: Will my child have physical limitations after surgery for osteosarcoma?

A: 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 that require partial or complete limb amputations will be fitted with a prosthesis and will need to undergo physical therapy to adapt to using it.

Q: Will my child need physical therapy?

A: Rehabilitation is an extremely important part of your child’s osteosarcoma care. This includes both physical and occupational therapy and 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.

Q: Will my child require an amputation?

A: Limb-sparing techniques such as limb-salvage surgery and rotationplasty allow our orthopedic surgeons to avoid amputation in many cases. However, if the tumor is very large or limb function cannot be preserved, your child’s physician may recommend amputation. If your child’s physician believes amputation is the best course of action, it is because other types of surgery may put your child at risk of relapse or leave them with a poorly functioning limb.

If amputation is necessary, your child will be fitted with a prosthesis through our NOPCO Brace Shop and will receive physical therapy to help them adjust.

Q: What kind of follow-up care will my child require?

A: In order to monitor recovery, check for relapse and receive physical therapy, your child will need frequent follow-up care.

Questions to ask your child’s doctor

After your child is diagnosed with osteosarcoma, you may feel overwhelmed with information. It can be easy to lose track of the questions that occur to you.

Lots of parents find it helpful to jot down questions as they arise – that way, when you talk to your child’s doctors, you can be sure that all of your concerns are addressed.
If your child is old enough, you may want to suggest that she write down what she wants to ask her health care provider, too.

Some of the questions you may want to ask include:

  • How long will my child need to be in the hospital?
  • What are the possible short and long-term complications of treatment? How will they be addressed?
  • What is the likelihood of cure?
  • What services are available to help my child and my family cope?

Tests

The first step in treating your child is forming an accurate and complete diagnosis.

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 tests:

  • 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

After we complete all necessary tests, our experts meet to review and discuss what they have learned about your child's condition. Then we will meet with you and your family to discuss the results and outline the best treatment options.

Treatment and care

We know how difficult a diagnosis of osteosarcoma can be, both for your child and for your whole family. That’s why our physicians are focused on family-centered care: From your first visit, you’ll work with a team of professionals who are committed to supporting all of your family’s physical and psychosocial needs. We’ll work with you to create a care plan that’s best for your child.

Our pediatric oncologists and orthopedic surgeons have a wealth of experience treating osteosarcoma. 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.

What are the treatments for osteosarcoma?

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.

Traditional treatments for osteosarcoma
Treatments for osteosarcoma may involve a combination of therapies including surgery, chemotherapy and, rarely, radiation. In most cases, children receive chemotherapy before surgery (neoadjuvant), a surgical procedure to remove the tumor and additional chemotherapy after surgery (adjuvant).

Surgery
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. Our physical therapists will work with your child to help him adjust to the prosthesis.
  • Rotationplasty is an operation that preserves the lower leg and uses the ankle as a knee joint—especially useful in very young children where limb length would be an issue. This operation may be performed when the tumor is very large and complete removal would be difficult.

Surgery is personalized to help your child achieve the best functional and cosmetic outcome and to allow them to remain active and healthy. Talk to your child’s 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

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 different 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.

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.

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, as an injection into the muscle or fat tissue
  • intravenously, directly to the bloodstream
  • intrathecally, directly into the spinal column with a needle

The chemotherapy used for osteosarcoma is given intravenously.

Radiation therapy
To treat some cancers, our doctors use precisely targeted and dosed radiation to kill cancer cells left behind after your child’s surgery. However, this is rarely used to treat osteosarcoma.

Rehabilitation
Rehabilitation is an extremely important part of your child’s osteosarcoma care. This includes both physical and occupational therapy and 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.

Unfortunately, some cases of osteosarcoma require partial or complete amputation of an arm or leg. At Boston Children's Hospital, our NOPCO Brace Shop provides each patient with an expertly-made, customized prosthesis and our physical therapists help children adjust to using them.

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

What is the recommended long-term care for children treated for osteosarcoma?

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

Through the David B. Perini, Jr. Quality of Life Clinic, childhood cancer survivors receive a comprehensive follow-up evaluation from their cancer care team.

  • Our childhood cancer survivorship clinic is held weekly.
  • In addition to meeting with your pediatric oncologists, your child may see one of our endocrinologists, cardiologists, neurologists, neuro-psychologists or alternative/complementary therapy specialists.
  • We also offer the following services:
    • patient and family education
    • psychosocial assessment
    • genetic counseling
    • reproductive and fertility evaluation and counseling
    • opportunities to speak with other childhood cancer survivors
     
What treatments are available for relapsed or recurrent osteosarcoma?

Unfortunately, about 30 percent of children treated for osteosarcoma will experience relapse, a return of the disease.

Our pediatric oncologists and surgeons are experienced in treating relapsed osteosarcoma. For children with relapsed disease, 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 and we are a Children’s Oncology Group site.

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

Your child’s chance of recovery and treatment options depend on a number of different factors:

  • 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. Prompt medical attention and appropriate therapy are important for the best prognosis.

Continual follow-up care is essential if your child has osteosarcoma. Side effects of surgery and chemotherapy, as well as recurrence of the disease, can occur in survivors of osteosarcoma.

Long-term follow-up

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.

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 in New England and elsewhere, to manage these long-term consequences.

Founded in 1972, Boston Children's Hospital's NOPCO Brace Shop provides orthotics and prosthetics for children treated for osteosarcoma.

Research and innovations

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

We are also 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 and chemotherapy to more effectively overcome cancer.

Our pediatric oncologists are involved in designing new trials through the Children’s Oncology Group. We also have a very active basic research program. We have a mouse model for osteosarcoma and we’re actively studying it to try to identify new potential treatments.

Research underway

We have available all Children’s Oncology Group clinical trials for newly diagnosed, non-metastatic and metastatic osteosarcoma. Here is some of the research that our researchers are currently working on:

  • We are examining whether an immune-modulating drug called interferon will improve outcomes for patients with osteosarcoma.
  • We studying whether the addition of 2 chemotherapy agents, ifosfamide and etoposide, to standard chemotherapy will help patients with high-risk osteosarcoma.
  • We are investigating whether an osteoporosis drug called zolotronic acid can improve the outcome of patients with metastatic osteosarcoma.
  • 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 in order to increase the effectiveness of treatment and prevent short- and long-term complications.

Osteosarcoma

Overview

Osteosarcoma occurs predominantly in adolescents and young adults.

Review of data from the Surveillance, Epidemiology and End Results program of the NCI resulted in an estimate of 4.4 per million new cases of osteosarcoma each year in people aged 0 to 24 years. The U.S. Census Bureau estimates that there will be 110 million people in this age range in 2010, resulting in an incidence of roughly 450 cases per year in children and young adults less than 25 years old.
  Osteosarcoma accounts for approximately 5% of childhood tumors. In children and adolescents, more than 50% of these tumors arise from the bones around the knee.

Osteosarcoma can rarely be observed in soft tissue or visceral organs. There appears to be no difference in presenting symptoms, tumor location, and outcome for younger patients (<10 years) compared with adolescents.


Two trials conducted in the 1980s were designed to address the natural history of surgically treated localized, resectable osteosarcoma of the extremity. The outcome of patients in these trials who were treated with surgical removal of the primary tumor recapitulated the historical experience before 1970; more than half of these patients developed metastases within 6 months of diagnosis, and overall, approximately 90% developed recurrent disease within 2 years of diagnosis.


Overall survival for patients treated with surgery alone was statistically inferior. The natural history of osteosarcoma has not changed over time, and fewer than 20% of patients with localized resectable primary tumors treated with surgery alone can be expected to survive free of relapse.
 

 

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