Bone cancer begins when cells in the bone begin to change, grow without control, and no longer die, forming a mass called a tumor. Bone cancer is rare and accounts for less than 1% of all new cancers. This section contains information about primary bone cancer (cancer that begins in the bone). It is much more common for bones to be the site of metastasis (spreading) from other cancers, such as breast, lung, or prostate cancer. For information about cancer that has started in another part of the body and spread to the bone, please see the information for that type of cancer.
The human skeletal system is made up of more than 200 bones that protect the internal organs, allow people to stand upright, and attach to muscles that allow movement. Bones are connected to other bones by ligaments, which are bands of tough, fibrous tissue. Cartilage is the tough, fibrous material that covers and protects the joints where bones come together. Bones are hollow and filled with bone marrow, which is spongy, red tissue that produces blood cells.
Bone is a tissue that consists of collagen (a soft, fibrous tissue) and calcium phosphate (a mineral that helps harden and strengthen the bone). There are three types of bone cells:
Osteoclasts break down and remove old bone.
Osteoblasts build new bone.
Osteocytes carry nutrients to the bone.
Cancer can occur in any of these parts of the bone. A bone tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body). Even though a benign tumor does not spread outside the bone, it can grow large enough to press on surrounding tissues and weaken the bone. A malignant tumor can destroy the cortex (the hard outer portion of the bone) and spread to nearby tissue. If bone tumor cells get into the bloodstream, they can spread to other parts of the body, especially the lungs.
There are different types of bone cancer. Osteosarcoma and Ewing’s sarcoma are two of the most common types of bone cancer and generally occur in children and young adults. Chondrosarcoma is cancer of the cartilage and is more common in adults. Chordoma is a type of bone cancer that typically starts in the lower spinal cord. Rare, soft-tissue sarcomas that begin in the bone include malignant fibrous histiocytoma (MFH) and fibrosarcoma. MFH makes up less than 1% of primary bone tumors. It is usually found in adults. The arms and legs, especially around the knee joint, are the most common sites for MFH to appear. Fibrosarcoma is also more common among adults, particularly during middle age, and most often begins in the thighbone. Paget’s disease of the bone generally occurs in older adults and involves the overgrowth of bony tissue.
Statistics
In 2008, an estimated 2,380 adults (1,270 men and 1,110 women) in the United States will be diagnosed with bone cancer. It is estimated that 1,470 deaths (820 men and 650 women) from this disease will occur this year. Primary bone cancer accounts for less than 0.2% of all cancers.
Osteosarcoma makes up 35% of all primary bone cancers, followed by chondrosarcoma (26%), Ewing’s family of tumors (16%), chordoma (8%), and MFH/fibrosarcoma (6%). There are other rare types of cancer that account for the remainder of cases.
Cancer survival statistics should be interpreted with caution. These estimates are based on data from thousands of cases of this type of cancer, 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 bone cancer. Because the survival statistics are measured in five-year (or sometimes one-year) intervals, they may not represent advances made in the treatment or diagnosis of this cancer.
Statistics adapted from the American Cancer Society's publication, Cancer Facts and Figures 2008.
A risk factor is anything that increases a person’s chance of developing cancer. Some risk factors can be controlled, such as smoking, and some cannot be controlled, such as age and family history. Although risk factors can influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and communicating them to your doctor may help you make more informed lifestyle and health-care choices.
The following factors may raise a person’s risk of developing bone cancer:
Genetics. Children with familial retinoblastoma (an eye cancer) have an increased risk of developing osteosarcoma.
Previous radiation therapy. People who have had radiation treatment for other conditions have a higher risk of developing bone cancer at the site of the radiation therapy. The majority of radiation therapy-caused bone cancers are osteosarcomas, but other types may occur.
Chemotherapy. Some drugs, including alkylating agents and anthracyclines, used to treat cancer may increase the risk of developing a secondary cancer, usually osteosarcoma.
Benign tumors or other bone conditions. Paget’s disease may cause osteosarcoma. Other benign bone diseases, such as fibrous dysplasia, may increase the risk of osteosarcoma.
Currently, there is no known way of preventing bone cancer. Early detection offers the best hope for successful treatment, so people with risk factors are encouraged to visit the doctor regularly. Still, most bone cancer occurs in people with no known risk factors.
People with bone cancer may experience the following symptoms. Sometimes, people with bone cancer do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom described below, please talk with your doctor.
When a bone tumor grows, it compresses normal tissue and destroys bone tissue, which can cause symptoms. The earliest symptoms of bone cancer are pain and swelling in the area of the tumor. The pain may come and go at first, then become more severe and steady later. The pain may worsen with movement, and there may be swelling in the soft tissue nearby. A tumor that occurs near or in joints may cause the joint to swell and become tender or stiff, which means a person may experience a limited and painful range of movement.
A pronounced limp (if the leg is affected) or a fracture (break) in the affected bone are symptoms of more advanced bone cancer. Rarely, symptoms such as fever, malaise (feeling unwell), weight loss, and anemia occur with bone cancer.
Doctors use many tests to diagnose cancer and determine if it has metastasized. Some tests may also determine which treatments may be the most effective. For most types of cancer, 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 metastasized. Benign and cancerous tumors usually look different on imaging tests. A benign tumor has round, smooth, well-defined borders. A cancerous tumor has irregular, poorly defined border due to aggressive growth. There may also be evidence of bone destruction on an image of a cancerous tumor. Imaging tests may suggest a diagnosis of bone cancer, but a biopsy will always be performed to confirm the diagnosis and determine the subtype. It is extremely important for a patient to be seen by a sarcoma specialist before any surgery or a biopsy is performed.
Your doctor may consider these factors when choosing a diagnostic test:
Age and medical condition
The type of cancer suspected
Severity of symptoms
Previous test results
In addition to a physical examination, the following tests may be used to diagnose bone cancer:
Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). The type of biopsy (needle or incisional) performed will depend on the location of the cancer. However, there are some cases where a biopsy may not be able to be performed.
For a needle biopsy, a small hole is made in the bone, and a tissue sample is removed from the tumor with a needle-like instrument. In an incisional biopsy, the tissue sample is removed after a small cut is made in the tumor.
Blood tests. Some laboratory tests may help detect bone cancer. Alkaline phosphatase and lactate dehydrogenase levels may be higher in patients with osteosarcoma or Ewing’s sarcoma. However, it is important to note that alkaline phosphatase is normally high when cells that form bone tissue are very active (for example, when children are growing or a broken bone is mending), so high levels do not always mean cancer. Patients with chondrosarcoma may have abnormal glucose tolerance tests.
X-ray. An x-ray is a picture of the inside of the body. Typically, if an x-ray suggests cancer, the doctor would order other imaging tests.
Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture 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. Sometimes, a contrast medium (a special dye) is injected into a vein to provide better detail.
Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture. MRI scans are useful to check for tumors in nearby soft tissues.
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 cancerous cells, appear dark.
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 radioactive substance is injected into a patient’s body and absorbed by the organs or tissues being studied. This substance gives off energy that is detected by a scanner, which produces the images.
Integrated PET-CT scan. This scanning method collects images from both CT and PET scans at the same time, and then combines the images. This technique has the advantage of looking at both the structure and metabolism of the tumor and normal tissue. This information can be helpful to plan treatment and determine the benefits of different treatments.
Staging is a way of describing a cancer, such as where it is located, if and where it has spread, and if it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer'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 (chance of recovery). There are different stage descriptions for different types of cancer.
One tool that doctors use to describe the stage is the TNM system. This system uses three criteria to judge the stage of the cancer: the tumor itself, the lymph nodes around the tumor, and if the tumor has spread to the rest of the body. The results are combined to determine the stage of cancer for each person. There are five stages: stage 0 (zero) and stages I through IV (one through four). The stage provides a common way of describing the cancer, so doctors can work together to plan the best treatments.
TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer:
How large is the primary tumor and where is it located? (Tumor, T)
Has the tumor spread to the lymph nodes? (Node, N)
Has the cancer metastasized to other parts of the body? (Metastasis, M)
Tumor. Using the TNM system, the “T” plus a letter or number (0 to 4) is used to describe the size and location of the tumor. Some stages are also divided into smaller groups that help describe the tumor in even more detail. Specific tumor stage information for bone cancer is listed below.
TX: The primary tumor cannot be evaluated.
T0: There is no evidence of a primary tumor.
T1: The tumor is 8 centimeters (cm) or smaller.
T2: The tumor is 8 cm or larger.
T3: There is more than one separate tumor in the primary bone site.
Node. The “N” in the TNM staging system stands for lymph nodes, the tiny, bean-shaped organs that help fight infection. Lymph nodes near the site of the cancer are called regional lymph nodes. Lymph nodes in other parts of the body are called distant lymph nodes. Spread to the regional lymph nodes is rare for primary bone cancer.
NX: The regional lymph nodes cannot be evaluated.
N0: The cancer has not spread to the regional lymph nodes.
N1: The cancer has spread to the regional lymph nodes.
Distant metastasis. The “M” in the TNM system indicates whether the cancer has spread to other parts of the body.
MX: Metastasis cannot be evaluated.
M0: The cancer has not metastasized.
M1: There is metastasis to another part of the body.
M1a: There is metastasis to the lung.
M1b: There is metastasis to another organ.
Grade. A cancer may also be graded, which describes how much cancer cells look like healthy cells under a microscope. If they look like healthy cells, called well differentiated, the cancer is a low-grade tumor. If they look very little like healthy cells, called poorly differentiated, the cancer is a high-grade tumor. A tumor’s grade is described using the letter “G” and a number. The grade of cancer can help the doctor predict how quickly the cancer will spread, with lower grade tumors having a generally better prognosis.
GX: The tumor grade cannot be identified.
G1: The cancer cells are well differentiated.
G2: The cancer cells are moderately differentiated.
G3: The cancer cells are poorly differentiated.
G4: The cancer cells are undifferentiated.
Cancer stage grouping
Doctors assign the stage of the cancer by combining the T, N, and M classifications. In general, patients with the best prognosis have:
T1 or T2 tumor
A lower grade tumor (G1, G2)
A tumor that is easily removed with surgery, such as those located in the arms or legs
A localized tumor that has not spread
Certain genetic abnormalities
Stage IA: The tumor is low grade (G1 or G2) and 8 cm or smaller (T1). It has not spread to lymph nodes or other parts of the body (N0, M0).
Stage IB: The tumor is low grade (G1 or G2) and 8 cm or larger (T2). It has not spread to lymph nodes or other parts of the body (N0, M0).
Stage IIA: The tumor is high grade (G3 or G4) and 8 cm or smaller (T1). It has not spread to lymph nodes or other parts of the body (N0, M0).
Stage IIB: The tumor is high grade (G3 or G4) and 8 cm or larger (T2). It has not spread to lymph nodes or other parts of the body (N0, M0).
Stage III: There are multiple tumors of any grade in the primary bone site (T3), but they have not spread to lymph nodes or other parts of the body (N0, M0).
Stage IVA: The tumor is of any size or grade and has spread to the lung(s) (any T, N0, M1a, and any G).
Stage IVB: The tumor is of any size or grade and has spread to the lymph nodes (any T, N1, any M, and any G), or the tumor is of any size or grade and has spread to another organ (any T, any N, M1b, any G).
Recurrent: Recurrent cancer is cancer that comes back after treatment.
Used with permission of the American Joint Committee on Cancer (AJCC), Chicago, Illinois. The original source for this material is the AJCC Cancer Staging Manual, Sixth Edition (2002) published by Springer-Verlag New York, www.springer-ny.com.
The treatment of bone cancer depends on the size and location of the tumor, whether the cancer has spread, and the patient’s overall health. In many cases, a team of doctors will work with the patient to determine the best treatment plan.
This section outlines treatments that are the standard of care (the best treatments available) for this specific type of cancer. Patients are also encouraged to consider clinical trials when making treatment plan decisions. A clinical trial is a research study to test a new treatment to prove it is safe, effective, and possibly better than standard treatment. Your doctor can help you review all treatment options. For more information, visit the Clinical Trials section.
For a low-grade tumor, the primary treatment is surgery. The goal of surgery is to remove the tumor and a margin of healthy bone or tissue around the tumor to make sure all the cancer cells are gone.
For a high-grade tumor, oncologists (a doctor who specializes in the care and treatment of people with cancer) often use a combination of treatments, including surgery, chemotherapy, and radiation therapy.
Surgery
Surgery for bone cancer often involves a wide excision of the tumor. A wide excision removes the tumor along with a margin of normal tissue in all directions. Limb-sparing techniques are used whenever possible. However, amputation (removal) of the affected arm or leg may be necessary depending on the tumor’s size or location.
Wide excision surgical techniques have reduced the number of amputations performed for patients with bone cancer. About 75% to 80% of patients can be treated with conservative surgery compared with amputation. These surgeries often require prostheses, such as metal plates or bone from other parts of the body, to replace the missing bone and provide strength to the remaining bone.
For some patients, amputation may offer the best option. These include patients whose cancer is located where it cannot be completely removed by surgery, patients who cannot undergo reconstruction, or when the surgical area cannot be adequately covered with soft tissue. Surgeons use soft tissue, such as muscle, to cover the reconstruction area. The tissue helps in healing and reduces the risk of infection.
Children with bone cancer may require amputation more often than adults since their bones grow more. To avoid amputation, some children can be fitted for expandable joint prostheses that adjust as the skeleton grows. These prostheses require multiple operations to adjust bone length as the child grows.
Chemotherapy
Chemotherapy is the use of drugs to kill cancer cells. Systemic chemotherapy is delivered through the bloodstream, targeting cancer cells throughout the body. Chemotherapy for bone cancer can usually be given as an outpatient treatment, which is treatment that can be given at a clinic or doctor’s office instead of being admitted to a hospital.
Chemotherapy is often useful for treating cancer that has metastasized. Fast-growing bone cancer may be treated first with chemotherapy before surgery. This often reduces the size of the primary tumor and may destroy tiny areas of metastasis if some of the cancer cells have spread to other areas.
Surgery alone is not usually sufficient treatment for patients with some bone cancers, particularly osteosarcoma. These cancers sometimes recur as distant metastases (most often in the lungs) that were most likely present in a microscopic form when the person was diagnosed. The use of chemotherapy has increased survival rates for some types of bone cancer.
For most high-grade tumors, the oncologist gives chemotherapy for three to four cycles before surgery to shrink the primary tumor, so the tumor is easier to remove. Chemotherapy before surgery may also improve survival, since it may kill cells that have spread from the original tumor. The tumor’s response to chemotherapy, evaluated with a microscope after the primary tumor has been removed, can be used to better determine the prognosis. Chemotherapy that is given before surgery is called preoperative chemotherapy, neoadjuvant chemotherapy, or induction chemotherapy.
After the patient has recovered from surgery, the patient may receive additional chemotherapy to kill any remaining tumor cells. This is called postoperative or adjuvant chemotherapy. The use of chemotherapy to shrink the tumor before surgery combined with chemotherapy after surgery has saved many lives and many patients’ limbs.
Some common chemotherapy drugs given to patients with bone cancer are ifosfamide (Ifex), methotrexate (multiple brand names), cyclophosphamide (Cytoxan, Neosar), etoposide (VePesid, Etopophos, Lastet), cisplatin (Platinol), doxorubicin (Adriamycin), and dactinomycin (Actinomycin-D, Cosmegen, Lyovac Cosmegen).
In particular, Ewing’s sarcoma responds well to chemotherapy. Some drugs used to treat Ewing’s sarcoma are vincristine (Oncovin), dactinomycin, cyclophosphamide, doxorubicin, ifosfamide, and etoposide.
The side effects of chemotherapy depend on the individual and the dose used, but can include fatigue, risk of infection, nausea and vomiting, loss of appetite, and diarrhea. These side effects usually go away once treatment is finished.
The medications used to treat cancer are continually being evaluated. Talking with your doctor is often the best way to learn about the medications prescribed for you, their purpose, and their potential side effects or interactions with other medications. Learn more about your prescriptions through Cancer.Net's Drug Information Resources, which provides links to searchable drug databases.
Radiation therapy
Radiation therapy is the use of high-energy x-rays or other particles to kill cancer cells. The most common type of radiation treatment is called external-beam radiation therapy, which is radiation given from a machine outside the body. When radiation treatment is given using implants, it is called internal radiation therapy or brachytherapy.
For bone cancer, radiation therapy is most often used for patients with a tumor that cannot be removed by surgery or that may have cancer cells remaining after surgery. Radiation therapy may be done before surgery to shrink the tumor, or it may be done after surgery to eliminate any cancer cells remaining after surgery. Radiation therapy makes it possible to do less extensive surgery, often preserving the arm or leg. Radiation therapy may also be used to alleviate pain in advanced bone cancer.
For patients with Ewing’s sarcoma, radiation therapy may be used with chemotherapy and surgery. However, oncologists have had good results in recent years using surgery for Ewing’s sarcoma, with or without radiation therapy. Ewing’s sarcoma that starts in bones that cannot be surgically removed is treated with chemotherapy and radiation therapy.
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.
Doctors and scientists are always looking for better ways to treat patients with bone cancer. A clinical trial is a way to test a new treatment to prove that it is safe, effective, and possibly better than a standard treatment. Patients who participate in clinical trials are 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.
Patients decide to participate in clinical trials for many reasons. For some patients, a clinical trial is the best treatment option available. Because standard treatments are not perfect, patients are often willing to face the added uncertainty of a clinical trial in the hope of a better result. Other patients volunteer for clinical trials because they know that finding new drugs and therapies is the only way to make progress in treating bone cancer. Even if they do not benefit directly from the clinical trial, their participation may benefit future patients with bone cancer.
To join a clinical trial, patients must complete a learning process known as informed consent. During informed consent, the doctor should list all of the patient’s options, so the person understands 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 standard treatment. Finally, the doctor must explain what will be required of each patient in order to participate in the clinical trial, including the number of doctor visits, tests, and the schedule of treatment. Learn more about clinical trials, including patient safety, phases of a clinical trial, deciding to participate in a clinical trial, questions to ask the research team, and links to find cancer clinical trials.
Cancer and its treatment can cause a variety of side effects. However, doctors have made major strides in recent years in reducing pain, nausea and vomiting, and other physical side effects of cancer treatments. Many treatments used today are less intensive but as effective as treatments used in the past. Doctors also have many ways to provide relief to patients when such side effects do occur.
Fear of treatment side effects is common after a diagnosis of cancer, but it may be helpful to know that preventing and controlling side effects is a major focus of your health-care team. Before treatment begins, talk with your doctor about possible side effects of the specific treatments you will be receiving. The specific side effects that can occur depend on a variety of factors, including the type of cancer, its location, the individual treatment plan (including the length and dosage of treatment), and the person’s overall health.
Ask your doctor which side effects are most likely to happen (and which are not), when side effects are likely to occur, and how they will be addressed by the health-care team if they do happen. Also, be sure to communicate with your doctor about side effects you experience during and after treatment. For more information on the most common side effects of cancer and different treatments, along with ways to prevent or control them, visit Cancer.Net’ s section on Managing Side Effects.
In addition to physical side effects, there may be psychosocial (emotional and social) effects as well. Learn more about the importance of addressing these needs in Cancer.Net’s section on Caring for the Whole Patient.
For more information on late effects or long-term side effects, please read the After Treatment section or talk with your doctor.
After treatment for bone cancer ends, talk with your doctor about developing a follow-up care plan. This plan may include regular physical examinations and/or medical tests to monitor your recovery for the coming months and years. For bone cancer, follow-up care typically includes general physical examinations, blood tests, and imaging studies (such as a bone scan, CT scan, or x-rays) to check for signs that the cancer has come back. Tell your doctor about any new symptoms, such as swelling or bone pain, because they may be signs that the cancer has come back or signs of other medical conditions.
Patients who undergo surgery for bone cancer, particularly amputation, often need physical therapy and other types of rehabilitative therapies. For more information, read Cancer Rehabilitation.
People recovering from bone cancer are encouraged to follow established guidelines for good health, such as maintaining a healthy weight, not smoking, eating a balanced diet, and having recommended cancer screening tests. Talk with your doctor to develop a plan that is best for your needs. For bone cancer survivors who smoke, quitting smoking may help recovery and reduce the risk of cancer recurrence. Learn more about Tobacco. Moderate exercise can help you rebuild your strength and energy level. Talk with your doctor about helping you create an appropriate exercise plan based upon your needs, physical abilities, and fitness level. Learn more about Healthy Living After Cancer.
Research for bone cancer is ongoing. The following advances may still be under investigation in clinical trials and may not be approved or available at this time. Always discuss all diagnostic and treatment options with your doctor.
Intraoperative radiation therapy. Clinical trials are evaluating the usefulness of radiation therapy given inside the body during surgery for some Ewing’s tumors. This is called intraoperative radiation therapy or internal radiation therapy.
Myeloablative therapy. A supplement to the treatment options for Ewing’s tumors is myeloablative therapy with stem cell support. Myeloablative therapy, an intense regimen of chemotherapy, destroys all cells that are dividing rapidly. This includes cancer cells but also some normal cells. Stem cells are cells that create all other types of cells in the body. They may be given to the patient after myeloablative therapy to boost the patient’s recovery.
Regular communication with your doctor is important in making informed decisions about your health care. Consider asking the following questions of your doctor:
What type of bone cancer do I have?
What stage is the bone cancer? What does that mean?
Where exactly is the cancer located?
Can you explain my pathology report (laboratory test results) to me?
What are my treatment options?
What clinical trials are open to me?
Which treatment option do you recommend? Why?
What are the possible side effects of this treatment, both in the short term and the long term?
What is the expected timeline for my treatment plan?
How will this treatment affect my daily life? Will I be able to work, exercise, and perform my usual activities?
What follow-up tests will I need, and how often will I need them?
What support services are available to me? To my family?
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