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Osteosarcoma - Childhood - Overview

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2014

ON THIS PAGE: You will find some basic information about this disease and the parts of the body it may affect. This is the first page of Cancer.Net’s Guide to Childhood Osteosarcoma. To see other pages, use the menu on the side of your screen. Think of that menu as a roadmap to this full guide.

About sarcoma

Sarcoma is cancer that develops in the tissues that support and connect parts of the body, including bone, fat, muscle, and soft tissue. Cancer begins when normal cells change and grow uncontrollably, forming a mass called a tumor. A tumor can be cancerous or benign. A cancerous tumor is malignant, meaning it can spread to other parts of the body. A benign tumor means the tumor will not spread.

About osteosarcoma

Osteosarcoma is a cancer of the bone that destroys tissue and weakens the bone. Osteosarcoma develops from immature bone cells that normally form new bone tissue. Osteosarcoma most often starts in the bones around the knee joint, either at the lower end of the thigh bone (femur) or at the upper end of the shin bone (tibia). The next most common place osteosarcoma begins is in the humerus, the upper arm bone close to the shoulder. However, osteosarcoma can develop in any bone in the body. Rarely, it can also occur as a tumor in the soft tissue of the body, outside the bone.

Osteosarcoma is described as either a medullary tumor, also called a central tumor, or a peripheral tumor, also called a surface tumor. Each has different subtypes. The type and subtype of osteosarcoma is determined by looking at the tumor cells through a microscope. The most common subtype is called conventional central osteosarcoma. The other subtypes are much less common, each accounting for less than 5% of all osteosarcomas.

Medullary osteosarcoma subtypes include:

  • Conventional central osteosarcoma
  • Telangiectatic osteosarcoma
  • Intraosseous well-differentiated, or low-grade, osteosarcoma
  • Small cell osteosarcoma

Peripheral osteosarcoma subtypes include:

  • Parosteal (juxtacortical) well-differentiated, or low-grade, osteosarcoma
  • Periosteal osteosarcoma that is low grade to intermediate grade
  • High-grade surface osteosarcoma

This section covers osteosarcoma that is diagnosed during childhood. To learn more about other types of bone cancer and soft-tissue sarcoma, visit their specific sections on Cancer.Net.

Looking for More of an Overview?

If you would like additional introductory information, explore these related items. Please note these links take you to other sections on Cancer.Net:

  • ASCO Answers Fact Sheet: Read a one-page fact sheet, available as a PDF, that offers an easy-to-print introduction to this type of cancer.
  • Cancer.Net Patient Education Video: View a short video led by an ASCO expert in childhood cancer that provides basic information and areas of research.

To continue reading this guide, use the menu on the side of your screen to select another section.

Osteosarcoma - Childhood - Statistics

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2014

ON THIS PAGE: You will find information about how many people, including children, learn they have this type of cancer each year and some general survival information. Remember, survival rates depend on several factors. To see other pages, use the menu on the side of your screen.

This year, an estimated 800 people total, including 400 people younger than 20, will be diagnosed with osteosarcoma in the United States. About 3% of all childhood cancers are osteosarcoma. It most often affects teenagers and young adults in their 20s.

The five-year survival rate is the percentage of people who survive at least five years after the cancer is detected, excluding those who die from other diseases. It depends on the type and subtype of the cancer, the cancer’s response to treatment, and the degree to which the cancer has spread. The five-year survival rate for children with osteosarcoma is about 70%.

Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of people with this type of cancer in the United States, but the actual risk for a particular individual may differ. It is not possible to tell a person how long he or she will live with osteosarcoma. Because the survival statistics are measured in five-year intervals, they may not represent advances made in the treatment or diagnosis of this cancer. Learn more about understanding statistics.

Statistics adapted from the American Cancer Society’s publication, Cancer Facts & Figures 2014.

To continue reading this guide, use the menu on the side of your screen to select another section.

Osteosarcoma - Childhood - Risk Factors

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2014

ON THIS PAGE: You will find out more about the factors that increase the chance of developing this type of cancer. To see other pages, use the menu on the side of your screen.

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of a tumor, most do not directly cause cancer. Some people with several risk factors never develop a tumor, while others with no known risk factors do.

Most osteosarcoma begins sporadically, which means it appears in people who have no other diseases and no family history of bone cancer. Osteosarcoma may start because of overactive bone cells. Possible changes in genes that lead to this overactivity are being studied in a small number of families where siblings have developed osteosarcoma. Such changes, while very uncommon, may help doctors better understand how osteosarcoma begins in people with no family history of the disease, since similar genetic changes may occur in their cancer cells.

Osteosarcoma is probably caused by a combination of genetic changes that together cause immature bone cells to become tumor cells instead of developing into healthy bone. A gene known as RB that is abnormal in many children with a rare type of eye cancer called retinoblastoma may also be associated with osteosarcoma. RB is a tumor suppressor gene that normally controls the growth of cells. When it is mutated, or changed, it can no longer control cell growth, and a tumor can form. In addition, many other changes occur in the genes of osteosarcoma cells.

While all osteosarcomas are rare, there are groups of people that are more likely to develop osteosarcoma:

  • Teenagers who are having a growth spurt are most likely to develop osteosarcoma. Researchers are looking for a link between rapid bone growth and tumor development.
  • Osteosarcoma is about 50% more common in boys than girls.
  • People who have received radiation treatment for other types of cancer are more likely to develop osteosarcoma. Also, higher doses of radiation treatment at a younger age increase the risk.
  • Children with an inherited form of retinoblastoma are more likely to develop osteosarcoma.
  • Children with Li-Fraumeni syndrome have a higher risk of sarcoma, brain cancer, breast cancer, leukemia, and adrenal cancer. Li-Fraumeni syndrome is a rare disorder of the p53 gene, which is responsible for getting rid of abnormal cells.
  • Children with Werner syndrome, a very rare disorder that may involve the WRN gene, have a higher risk of sarcoma, thyroid cancer, and melanoma.
  • People with Rothmund-Thomson syndrome, an uncommon disorder characterized by short stature, or height; rash; hair loss called alopecia; and skeletal dysplasia, which are noncancerous abnormalities of the bones, are more likely to develop osteosarcoma.

To continue reading this guide, use the menu on the side of your screen to select another section.

Osteosarcoma - Childhood - Symptoms and Signs

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2014

ON THIS PAGE: You will find out more about body changes and other things that can signal a problem that may need medical care. To see other pages, use the menu on the side of your screen.

The symptoms of osteosarcoma depend on the bone in which the tumor developed. Children and teens with osteosarcoma may experience the following symptoms or signs. Sometimes, people with osteosarcoma do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer.

  • Pain in a bone or joint that gets worse over time
  • Painless swelling or a noticeable mass or lump in an arm or leg
  • A broken bone with no injury to explain how it occurred
  • Stiffness or swelling of joints; not a common symptom
  • Back pain or a loss of bowel or bladder control, which can occur if the tumor is in the pelvis or at the base of the spine; not a common early symptom

If you are concerned about one or more of the symptoms or signs on this list, please talk with your child’s doctor. Your doctor will ask how long and how often your child has been experiencing the symptom(s), in addition to other questions. This is to help find out the cause of the problem, called a diagnosis.

If cancer is diagnosed, relieving symptoms remains an important part of cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your child’s health care team about symptoms your child experiences, including any new symptoms or a change in symptoms.

The next section helps explain what tests and scans may be needed to learn more about the cause of the symptoms. Use the menu on the side of your screen to select Diagnosis, or you can select another section, to continue reading this guide.

Osteosarcoma - Childhood - Diagnosis

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2014

ON THIS PAGE: You will find a list of the common tests, procedures, and scans that doctors can use to find out what’s wrong and identify the cause of the problem. To see other pages, use the menu on the side of your screen.

Doctors use many tests to diagnose a tumor and find out if it has metastasized, meaning the tumor has spread from where it began to another part of the body. Some tests may also determine which treatments may be the most effective. For most tumors, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis. Imaging tests may be used to find out whether the cancer has spread. This list describes options for diagnosing this type of cancer, and not all tests listed will be used for every child. Your child’s doctor may consider these factors when choosing a diagnostic test:

  • Age and medical condition
  • Type of tumor suspected
  • Signs and symptoms
  • Previous test results

In addition to a physical examination, the following tests may be used to diagnose osteosarcoma. It is recommended that a health care team with experience diagnosing and treating bone tumors perform these tests.

Imaging tests

X-ray. An x-ray is a way to create a picture of the structures inside of the body using a small amount of radiation. The doctor will take an x-ray of the area where there is a lump or swelling. Osteosarcoma usually shows certain common features on an x-ray.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. A CT scan can also be used to measure the tumor’s size. Sometimes, a special dye called a contrast medium is given before the scan to provide better detail on the image. This dye can be injected into a patient’s vein or given as a pill to swallow.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. MRI can also be used to measure the tumor’s size.  A special dye called a contrast medium is given before the scan to create a clearer picture. This dye can be injected into a patient’s vein or given as a pill to swallow. An MRI creates more detailed pictures than CT scans and can sometimes help find a smaller tumor. Also, an MRI provides more exact pictures of the tumor and the surrounding healthy tissue. This can help the orthopedic surgeon, a doctor who specializes in surgery on the bones, plan surgery to remove the tumor-containing area of bone with a portion of the surrounding healthy tissue called the margin. 

Positron emission tomography (PET) scan. A PET scan is a way to create pictures of organs and tissues inside the body. A small amount of a radioactive sugar substance is injected into the patient’s body. This sugar substance is taken up by cells that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body.

Bone scan. A bone scan uses a radioactive tracer to look at the inside of the bones. The tracer is injected into a patient’s vein. It collects in areas of the bone and is detected by a special camera. Healthy bone appears gray to the camera, and areas of injury, such as those caused by a tumor, appear dark. However, it is also normal that areas where new bone is being formed, such as the growth plates, known as epiphyses, appear dark.

Arteriogram (also called angiogram). An arteriogram is a way for doctors to see inside the arteries. A small amount of a contrast medium is injected into an artery, making it appear on an x-ray. A surgeon may use this test to help plan surgery.

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that a tumor is present, but only a biopsy can make a definitive diagnosis. The sample removed during the biopsy is analyzed by a pathologist. A pathologist is a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease. A doctor who specializes in bone tumors should perform the biopsy, which typically involves surgery, or sometimes, a needle biopsy. A needle biopsy uses a hollow needle inserted into the tumor. Doctors may analyze the genes in the cancer cells to distinguish osteosarcoma from other types of cancer.

After diagnostic tests are done, your child’s doctor will review all of the results with you. If the diagnosis is cancer, these results also help the doctor describe the location(s) of the cancer; this is called staging.

The next section helps explain the different stages for this type of cancer. Use the menu on the side of your screen to select Stages, or you can select another section, to continue reading this guide.

Osteosarcoma - Childhood - Stages

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2014

ON THIS PAGE: You will learn about how doctors describe a cancer’s growth or spread. This is called the stage. To see other pages, use the menu on the side of your screen.

Staging is a way of describing where the tumor is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to find out the tumor's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis, which is the chance of recovery. There are different stage descriptions for different tumors.

The terms doctors use to describe the stage of osteosarcoma and plan treatment include:

Localized: The tumor is only in the bone where it began and in the tissue around it. The tumor has not spread to other parts of the body.

Metastatic: The tumor has spread from the bone where it began to another part of the body, most often the lungs or other bones.

Recurrent: Recurrent osteosarcoma is a tumor that has come back either during or after treatment. It can come back in the same place where it started or in another part of the body. Osteosarcoma recurs most often in the lungs and other bones. If there is a recurrence, the cancer may need to be staged again (called re-staging) using the system above.

Source: National Cancer Institute.

Information about the cancer’s stage will help the doctor recommend a treatment plan. The next section helps explain the treatment options for this type of cancer. Use the menu on the side of your screen to select Treatment Options, or you can select another section, to continue reading this guide.

Osteosarcoma - Childhood - Treatment Options

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2014

ON THIS PAGE: You will learn about the different ways doctors use to treat children and teens with this type of cancer. To see other pages, use the menu on the side of your screen.

In general, cancer in children is uncommon, so it can be hard for doctors to plan treatments unless they know what has been most effective in other children. That is why more than 60% of children with cancer are treated as part of a clinical trial. Clinical trials are research studies that compare the standard treatments (the best proven treatments available) with newer approaches to treatments that may be more effective. Clinical trials may test such approaches as a new drug, a new combination of standard treatments, or new doses of current therapies. Studying new treatments involves careful monitoring using scientific methods, and all participants are followed closely to track their health and progress.

To take advantage of these newer treatments, all children with cancer should be treated at a specialized cancer center. Doctors at these centers have extensive experience in treating children with cancer and have access to the latest research. A doctor who specializes in treating children with cancer is called a pediatric oncologist. In many cases, a team of doctors works with a child and the family to provide care; this is called a multidisciplinary team. Pediatric cancer centers often have extra support services for children and their families, such as child life specialists, nutritionists, physical and occupational therapists, social workers, and counselors. Special activities and programs to help your child and family cope may also be available.

Descriptions of the most common treatment options for osteosarcoma are listed below. The three most common types of treatment for osteosarcoma are surgery, chemotherapy, and radiation therapy. Treatment options and recommendations depend on several factors, including the type and stage of cancer, possible side effects, the child’s overall health, and the child’s and family’s preferences. Your child’s care plan may also include treatment for symptoms and side effects, an important part of cancer care. Take time to learn about your child’s treatment options and be sure to ask questions about things that are unclear. Also, talk about the goals of each treatment with your child’s doctor and what your child can expect while receiving the treatment. Learn more about making treatment decisions.

Surgery

Surgery is the removal of the tumor and some surrounding tissue during an operation. An orthopedic oncologist is a doctor who specializes in surgery when cancer affects the bone. Chemotherapy and, occasionally, radiation therapy may be used with surgery, particularly with limb-sparing surgery. In limb-sparing surgery, doctors use surgical techniques, such as bone grafting and reconstructive surgery, to help the child keep the use of the arm or leg and to give the limb a more normal appearance. Limb-sparing surgery is used whenever possible. Sometimes, the operation that results in the most useful and strongest limb is different from the one that gives the most normal appearance. Learn more about cancer surgery.

Occasionally, a limb will need to be amputated, or removed, to be sure all of the tumor has been eliminated. If amputation is needed, rehabilitation, including physical therapy, can help the child maximize his or her physical functioning. Rehabilitation can also help a child cope with the social and emotional effects of losing a limb. Learn more about rehabilitation.

Chemotherapy

Chemotherapy is the use of drugs to destroy cancer cells, usually by stopping the cancer cells’ ability to grow and divide. Chemotherapy is given by a pediatric or medical oncologist, a doctor who specializes in treating cancer with medication.

Systemic chemotherapy is delivered through the bloodstream to reach tumor cells throughout the body. Common ways to give chemotherapy include an intravenous (IV) tube placed into a vein using a needle or in a pill or capsule that is swallowed (orally). Chemotherapy for osteosarcoma is usually given intravenously. Occasionally, medications are taken by mouth or injected under the skin, usually to limit the side effects of treatment. A chemotherapy regimen (schedule) usually consists of a specific number of cycles given over a set period of time. Your child may receive one drug at a time or a combination of different drugs at the same time.

Chemotherapy is often given before surgery to reduce tumor size and avoid amputation of the arm or leg. Also, it is almost always given after surgery to destroy any tumor cells that may still be in the body.

The side effects of chemotherapy depend on the individual and the dose used, but they can include fatigue, risk of infection, nausea and vomiting, hair loss, loss of appetite, and diarrhea. These immediate side effects can be managed by the health care team during treatment and usually go away once treatment is finished. Other side effects, such as decreased strength of the heart muscle, hearing loss, or decreased kidney function, may continue after treatment. The severity of the side effects depends on the type of drug given and the length of treatment. Learn more about long-term side effects in the After Treatment section. 

Learn more about chemotherapy and preparing for treatment. The medications used to treat cancer are continually being evaluated. Talking with your child’s doctor is often the best way to learn about the medications prescribed for your child, their purpose, and their potential side effects or interactions with other medications. Learn more about your child’s prescriptions by using searchable drug databases.

Radiation therapy

Radiation therapy is the use of high-energy x-rays or other particles to destroy cancer cells. A doctor who specializes in giving radiation therapy to treat cancer is called a radiation oncologist. The most common type of radiation treatment for osteosarcoma is called external-beam radiation therapy, which is radiation therapy given from a machine outside the body. A radiation therapy regimen (schedule) usually consists of a specific number of treatments given over a set period of time.

Radiation therapy for osteosarcoma is uncommon and is only used when trying to avoid amputating an arm or leg. In fact, because of osteosarcoma’s relative resistance to radiation, radiation therapy is not often used in osteosarcoma. Surgery and chemotherapy are the most common types of treatment. However, this situation may change with the increased ability to deliver higher doses of radiation safely to just about any part of body.

Side effects from radiation therapy may include fatigue, mild skin reactions, upset stomach, and loose bowel movements. Most side effects go away soon after treatment is finished. Learn more about radiation therapy.

Getting care for symptoms and side effects

Cancer and its treatment often cause side effects. In addition to treatment to slow, stop, or eliminate the cancer, an important part of cancer care is relieving a person’s symptoms and side effects. This approach is called palliative or supportive care, and it includes supporting the patient with his or her physical, emotional, and social needs.

Palliative care can help a person at any stage of illness. People often receive treatment for the cancer and treatment to ease side effects at the same time. In fact, patients who receive both often have less severe symptoms, better quality of life, and report they are more satisfied with treatment.

Palliative treatments vary widely and often include medication, nutritional changes, relaxation techniques, and other therapies. Your child may also receive palliative treatments similar to those meant to eliminate the cancer, such as chemotherapy, surgery, and radiation therapy. Talk with your child’s doctor about the goals of each treatment in the treatment plan.

Before treatment begins, talk with your child’s health care team about the possible side effects of the recommended treatment plan and supportive care options. And during and after treatment, be sure to tell your child’s doctor or another health care team member if your child experiences a problem so it is addressed as quickly as possible. Learn more about palliative care.

Remission and the chance of recurrence

A remission is when cancer cannot be detected in the body. This may also be called having “no evidence of disease” or being “NED.” 

A remission may be temporary or permanent. This uncertainty causes many childhood cancer survivors and their families to feel worried or anxious that the cancer will come back. While many remissions are permanent, it is important to talk with your doctor about the possibility of the cancer returning. Understanding the risk of recurrence and the treatment options may help you and your child feel more prepared if the cancer does return. Learn more about coping with the fear of recurrence

If the cancer does return after the original treatment, it is called recurrent cancer. It may come back in the same place (called a local recurrence), nearby (regional recurrence), or in another place (distant recurrence).

When this occurs, a cycle of testing will begin again to learn as much as possible about the recurrence, including whether the cancer’s stage has changed. After testing is done, you and your child’s doctor will talk about the treatment options. Often the treatment plan will include the therapies described above, such as surgery and chemotherapy, but they may be used in a different combination or given at a different pace. Your child’s doctor may also suggest clinical trials that are studying new ways to treat this type of recurrent cancer.

Treatment for recurrent osteosarcoma depends on three factors:

  • Where the cancer recurred
  • The type of treatment your child received for the original tumor
  • The overall health of your child

If the disease recurs in the lungs, surgery is often used to remove the nodules, or tumors, in the lung. In these situations, there is usually a better outcome for children who have the lung nodules completely removed. This is especially true if the disease has recurred only after the initial treatment has been completed. Chemotherapy or other approaches may be used as well. If the cancer comes back elsewhere in the body, a combination of drugs may be used. If the cancer has spread to another bone or to a small number of other bones, surgery may be performed, particularly if chemotherapy has worked well.

When cancer recurs, patients and their families often experience emotions such as disbelief or fear. Families are encouraged to talk with their health care team about these feelings and ask about support services to help them cope. Learn more about dealing with cancer recurrence.

If treatment fails

Although treatment is successful for the majority of children with cancer, sometimes it is not. If a child’s cancer cannot be cured or controlled, this is called advanced or terminal cancer. This diagnosis is stressful, and it may be difficult to discuss. However, it is important to have open and honest conversations with your child’s doctor and health care team to express your family’s feelings, preferences, and concerns. The health care team is there to help, and many team members have special skills, experience, and knowledge to support patients and their families.

Parents or guardians are encouraged to think about where the child would be most comfortable: at home, in a home-like setting elsewhere, in the hospital, or in a hospice environment. Hospice care is a type of palliative care for people who are expected to live less than six months. It is designed to provide the best possible quality of life for people who are near the end of life. Nursing care and special equipment can make staying at home a workable alternative for many families. Some children and teens may be happier if they can arrange to attend school part-time or keep up other activities and social connections. The child’s health care team can help parents or guardians decide on an appropriate level of activity. Making sure a child is physically comfortable and free from pain is extremely important as part of end-of-life care. Learn more about caring for a terminally ill child and advanced cancer care planning.

The death of a child is an enormous tragedy, and families may need support to help them cope with the loss. Pediatric cancer centers often have professional staff and support groups to help with the process of grieving. Learn more on grieving the loss of a child.

The next section helps explain clinical trials, which are research studies. Use the menu on the side of your screen to select About Clinical Trials, or you can select another section, to continue reading this guide.

Osteosarcoma - Childhood - About Clinical Trials

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2014

ON THIS PAGE: You will learn more about clinical trials, which are the main way that new medical approaches are tested to see how well they work. To see other pages, use the menu on the side of your screen.

Doctors and scientists are always looking for better ways to treat children and teens with osteosarcoma. To make scientific advances, doctors create research studies involving volunteers, called clinical trials.

Many clinical trials are focused on new treatments, evaluating whether a new treatment is safe, effective, and possibly better than the current (standard) treatment. These types of studies evaluate new drugs, different combinations of existing treatments, new approaches to radiation therapy or surgery, and new methods of treatment. Children and teens who participate in clinical trials are often among the first to receive new treatments before they are widely available. However, there is no guarantee that the new treatment will be safe, effective, or better than a standard treatment.

There are also clinical trials that study new ways to ease symptoms and side effects during treatment and manage the late effects that may occur after treatment. Talk with your child’s doctor about clinical trials regarding side effects.

People decide to participate in clinical trials for many reasons. For some, a clinical trial is the best treatment option available. Because standard treatments are not perfect, people are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other people volunteer for clinical trials because they know that these studies are the only way to make progress in treating osteosarcoma. Even if they do not benefit directly from the clinical trial, their participation may benefit future children and teens with osteosarcoma.

Sometimes people have concerns that, by participating in a clinical trial, they may receive no treatment by being given a placebo or a “sugar pill.” The use of placebos in cancer clinical trials is rare. When a placebo is used in a study, it is done with the full knowledge of the participants. Find out more about placebos in cancer clinical trials.

To join a clinical trial, parents and children must participate in a process known as informed consent. During informed consent, the doctor should list all of the child’s options, so that the parent and child understand how the new treatment differs from the standard treatment. The doctor must also list all of the risks of the new treatment, which may or may not be different than the risks of the standard treatment. Finally, the doctor must explain what will be required of each child in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment.

For specific topics being studied for osteosarcoma, learn more in the Latest Research section.

Patients who participate in a clinical trial may stop participating at any time for any personal or medical reason. This may include that the new treatment is not working or there are serious side effects. Clinical trials are also closely monitored by experts who watch for any problems with each study. It is important that parents of a child participating in a clinical trial talk with their child’s doctor and the researchers about who will be providing their child’s treatment and care during the clinical trial, after the clinical trial ends, and/or if they choose to leave the clinical trial before it ends. 

Cancer.Net offers a lot of information about cancer clinical trials in other areas of the website, including a complete section on clinical trials and places to search for clinical trials for a specific type of cancer.

The next section helps explain the areas of research going on today about this type of cancer. Use the menu on the side of your screen to select Latest Research, or you can selection another section, to continue reading this guide.

Osteosarcoma - Childhood - Latest Research

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2014

ON THIS PAGE: You will read about the scientific research being done now to learn more about this type of cancer and how to treat it. To see other pages, use the menu on the side of your screen.

Doctors are working to learn more about osteosarcoma, how to best treat it, and how to provide the best care to children and teens diagnosed with this disease. The following areas of research may include new options for patients through clinical trials. Always talk with your child’s doctor about the diagnostic and treatment options best for your child.

Improved detection. Two types of imaging tests are being studied that may improve the detection of metastases: total body MRI and positron emission tomography (PET) scanning, which are described in the Diagnosis section. These tests can suggest the presence of metastatic disease, although other tests will be needed to confirm this suspicion. Specialists familiar with using these tests must interpret the results of the images. A biopsy may also be needed.

Supportive care. Clinical trials are underway to find better ways of reducing symptoms and side effects of current osteosarcoma treatments in order to improve patients’ comfort and quality of life.

Improved treatment. In several studies, researchers are looking at adding different drugs to standard treatment that may improve the treatment’s success without increasing the side effects. As part of the Children’s Oncology Group AOST 0331 study, pegylated interferon alpha was added after eight months of chemotherapy for patients with localized osteosarcoma or metastases to the lungs or bones that can be surgically removed, and whose tumor was almost completely eliminated by the initial 10 to 12 weeks of chemotherapy treatment. The preliminary results of this study showed that the addition of pegylated interferon alpha caused no significant improvements compared with chemotherapy alone. More information will become available as patients are followed for longer periods of time.

As part of a recent study, etoposide (VePesid, Toposar) and ifosfamide (Ifex) were added to treatment after surgery for a total of ten months of treatment instead of the standard eight months for patients with osteosarcoma that did not respond as well to initial treatment. According to the results, the additional therapy caused more side effects and did not improve the outcome of treatment. Therefore, the more intensive chemotherapy is not recommended. The Children’s Oncology Group considers the combination of cisplatin, doxorubicin, and high-dose methotrexate (multiple brand names) to be the standard treatment. There are other combination therapies that are similarly effective, but none of them is superior. 

A study for patients with initially metastatic disease has also recently been completed. It included a bone-stabilizing drug called zoledronic acid (Zometa) added to standard chemotherapy. It showed that the combination did not increase general side effects, which was the goal of the study. A French group studied whether the addition of zoledronic acid to chemotherapy for newly diagnosed patients with osteosarcoma would improve the outcome of treatment. Half of the patients received a standard chemotherapy treatment and surgery, and the other half received zoledronic acid as well. No additional improvements were seen in the group who received zoledronic acid. 

Another drug that is currently being tested is the immunotherapy mifamurtide (liposomal muramyl tripeptide phosphatidyl ethanolamine [L-MTP-PE] or MEPACT). Immunotherapy, also called biologic therapy, is designed to boost the body's natural defenses to fight the cancer. Mifamurtide is currently licensed in Europe by the European Medicines Association for the treatment of localized osteosarcoma that can be removed with surgery. However, it has not been approved by the U.S. Food and Drug Administration because the agency feels more research is needed to prove the drug’s effectiveness.

For information about these and other studies, visit the Children’s Oncology Group website. There are clinical trials using new drugs for patients with recurrent osteosarcoma—whether the cancer has come back a first, second, or subsequent time; whether the recurrence is local or distant; and whether the recurrence is located in the lungs, other bones, or both.

Talk with your child’s doctor for more information about clinical trials. Your doctor can provide additional details concerning the availability of these diagnostic tests or treatments, or others that are being studied. Also, your doctor can provide details on whether they are appropriate for your child.

Looking for More about the Latest Research?

If you would like additional information about the latest areas of research regarding osteosarcoma, explore these related items that will take you outside of this guide:

  • To find clinical trials specific to your diagnosis, talk with your child’s doctor or search online clinical trial databases now.
  • Visit ASCO’s CancerProgress.Net website to learn more about the historical pace of research for Childhood Cancers (called Pediatric Cancers on this website). Please note this link takes you to a separate ASCO website.

The next section addresses how to cope with the symptoms of the disease or the side effects of its treatment. Use the menu on the side of your screen to select Coping with Side Effects, or you can select another section, to continue reading this guide. 

Osteosarcoma - Childhood - Coping with Side Effects

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2014

ON THIS PAGE: You will find out more about steps to take to help your child cope with physical, social, and emotional side effects. This page includes several links outside of this guide to other sections of this website. To see other pages, use the menu on the side of your screen.

Fear of treatment side effects is common after a diagnosis of cancer, but it may help to know that preventing and controlling side effects is a major focus of your child’s health care team. This is called palliative or supportive care, and it is an important part of the overall treatment plan, regardless of the stage of disease.

Common side effects from each treatment option for osteosarcoma are described in detail within the Treatment Options section. Learn more about the most common side effects of cancer and different treatments, along with ways to prevent or control them. Side effects depend on a variety of factors, including the cancer’s stage, the length and dosage of treatment(s), and your child’s overall health.

Before treatment begins, talk with your child’s doctor about possible side effects of each type of treatment your child will be receiving. Ask which side effects are most likely to happen, when they are likely to occur, and what can be done to prevent or relieve them. And, ask about the level of caregiving your child may need during treatment and recovery, as family members and friends play an important role in the care of a child with osteosarcoma. Learn more about caregiving.

In addition to physical side effects, there may be emotional and social effects as well. Patients and their families are encouraged to share their feelings with a member of their health care team who can help with coping strategies, including concerns about managing the cost of cancer care

During and after treatment, be sure to tell the health care team about the side effects your child experiences, even if you feel they are not serious. Sometimes, side effects can last beyond the treatment period, called a long-term side effect. A side effect that occurs months or years after treatment is called a late effect. Treatment of both types of effects is an important part of survivorship care. Learn more by reading the After Treatment section or talking with your child’s doctor.

The next section helps explain medical tests and check-ups needed after finishing cancer treatment. Use the menu on the side of your screen to select After Treatment, or you can select another section, to continue reading this guide.

Osteosarcoma - Childhood - After Treatment

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2014

ON THIS PAGE: You will read about your child’s medical care after cancer treatment is finished and why this follow-up care is important. To see other pages, use the menu on the side of your screen.

After treatment for osteosarcoma ends, talk with your child’s doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your child’s recovery for the coming months and years. All children and teens treated for osteosarcoma should have life-long, follow-up care.

There are two major considerations for the long-term health of children and teens who have had osteosarcoma. The first is whether the surgery resulted in a well-functioning limb or whether there were complications. The most common orthopedic complications are fractures, which are commonly referred to as bone breaks, if bone from another person (a bone allograft) has been used; metallic failure or loosening if an internal prosthesis, such as an artificial knee, has been inserted; or infection. These complications can usually be treated with another surgery and, if there is an infection, long-term antibiotic therapy. Occasionally, these approaches fail and an amputation is needed.

The second consideration is whether there is a recurrence or late effects. Recurrence of the tumor more than five years later is rare. The drugs used to treat osteosarcoma have a small chance, approximately 1.5%, of causing a blood cancer called leukemia. This is called secondary or induced leukemia. Other possible late effects are related to the type of chemotherapy used. The most common drugs and long-term effects from them are:

Chemotherapy drug used

Potential long-term effect

Monitoring and management

Cisplatin (Platinol)

Hearing loss, neuropathy (pain or numbness in fingers and toes)

Monitored by hearing tests; hearing aids are sometimes needed.

Doxorubicin (Adriamycin)

Heart failure

Monitored most frequently with periodic echocardiograms

Etoposide

Induced leukemia

Treated similarly to a newly diagnosed person with leukemia depending on the type.

Ifosfamide

Infertility,

kidney damage, memory impairment

Freezing of sperm is recommended for boys who have gone through puberty before beginning chemotherapy. When it becomes a standard technique, ovarian cryopreservation, the freezing of a portion of the ovary, could be recommended for girls. Learn more about preserving fertility in children with cancer.

Kidney damage, especially loss of salts in the urine, may occur and requires supplements, but this is unlikely to develop if it has not already been a problem during treatment.

Methotrexate

Scarring of the lungs or liver, memory impairment

Breathing function tests, blood tests

Generally, most children and teens recovering from osteosarcoma do well. Based on the type of treatment your child received, the doctor will determine what examinations and tests are needed to check for long-term side effects, such as heart failure, hearing loss, kidney damage and the possibility of secondary cancers. Follow-up care should also address your child’s quality of life, including any developmental or emotional concerns, especially if amputation was necessary. Learn more about childhood cancer survivorship.

You and your family are encouraged to organize and keep a record of your child’s medical information. That way as your child enters adulthood, he or she has a clear, written history of the treatment given and the doctor’s recommendations about the schedule for follow-up care. The doctor’s office can help you create this. This information will be valuable to doctors who care for your child during his or her lifetime. ASCO offers treatment summary forms to help keep track of the treatment your child received and develop a survivorship care plan once treatment is completed.

Children and teens who have had cancer can also enhance the quality of their future by following established guidelines for good health into and through adulthood, including not smoking, maintaining a healthy weight, eating a balanced diet, and participating in regular physical activity. Talk with the doctor about developing a plan that is best for your child’s needs. Learn more about the next steps to take in survivorship.

The next section offers a list of questions you may want to ask. Use the menu on the side of your screen to select Questions to Ask the Doctor, or you can select another section, to continue reading this guide.

Osteosarcoma - Childhood - Questions to Ask the Doctor

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2014

ON THIS PAGE: You will find some questions to ask your child’s doctor or other members of your child’s health care team, to help you and your family better understand your child’s diagnosis, treatment plan, and overall care. To see other pages, use the menu on the side of your screen.

Talking often with the doctor is important to make informed decisions about your child’s health care. These suggested questions are a starting point to help you learn more about your child’s cancer care and treatment. You are also encouraged to ask additional questions that are important to you and your family. You may want to print this list and bring it to your child’s next appointment, or download Cancer.Net’s free mobile app for an e-list and other interactive tools to manage your child’s care.

Before a biopsy:

  • How experienced is the surgeon in doing this type of biopsy?
  • If the diagnosis is bone cancer, is the surgeon part of a team that is experienced in treating people with bone cancer? If not, can you or the surgeon refer me to a team?

After a biopsy:

  • Can you explain my child’s pathology report (laboratory test results) to me?
  • What type and subtype of osteosarcoma have been diagnosed? What does this mean?
  • Is the pathologist experienced in the diagnosis of osteosarcoma? And, is the diagnosis certain?
  • Is the disease located only in the bone where it started?
  • What are my child’s treatment options?
  • What clinical trials are open to my child? Where are they located, and how do I find out more about them?
  • What treatment plan do you recommend? Why?
  • What is the goal of each treatment? Is it to eliminate the cancer, help my child feel better, or both?
  • Who will be part of my child’s health care team, and what does each member do?
  • Who will be coordinating my child’s overall treatment and follow-up care?
  • Where will treatment take place?
  • Does this cancer center specialize in the treatment of children, adolescents, and young adults with cancer?
  • What are the possible side effects of this treatment, both in the short term and the long term?
  • How will this treatment affect my child’s daily life? Will he or she be able to go to school and perform his or her usual activities?
  • Could this treatment affect my child’s ability to become pregnant or have children? If so, should my family talk with a fertility specialist before cancer treatment begins?
  • What follow-up tests will my child need, and how often will he or she need them?
  • If I’m worried about managing the costs related to my child’s cancer care, who can help me with these concerns?
  • What support services are available to my child? To my family?
  • Whom should I call for questions or problems?

The next section offers some more resources that may be helpful to you. Use the menu on the side of your screen to select Additional Resources, or you can select another section, to continue reading this guide.

Osteosarcoma - Childhood - Additional Resources

This section has been reviewed and approved by the Cancer.Net Editorial Board, 08/2014

ON THIS PAGE: You will find some helpful links to other areas of Cancer.Net that provide information about cancer care and treatment. This is the final page of Cancer.Net’s Guide to Childhood Osteosarcoma. To go back and review other pages, use the menu on the side of your screen.

Cancer.Net includes many other sections about the medical and emotional aspects of cancer, both for the person diagnosed and their family members and friends. This website is meant to be a resource for you and your loved ones from the time of diagnosis, through treatment, and beyond. Here are a few sections that may get you started in exploring the rest of Cancer.Net:

This is the end of Cancer.Net’s Guide to Childhood Osteosarcoma. Use the menu on the side of your screen to continue reading this guide.