Bone cancer is very rare in adults. It starts in the cells that make up the bone. Cancer starts when cells begin to grow out of control. Cells in nearly any part of the body can become cancer, and can spread to other parts of the body. To learn more about how cancers start and spread, see What Is Cancer?

Normal bone tissue

To understand bone cancer, it helps to understand a little about normal bone tissue.

Survival rates are often used by doctors as a standard way of discussing a person’s prognosis (outlook). Some people with cancer may want to know the survival statistics for people in similar situations, while others may not find the numbers helpful, or may even not want to know them.

The 5-year survival rate refers to the percentage of patients who live at least 5 years after their cancer is diagnosed. Of course, many people live much longer than 5 years (and many are cured).

Five-year relative survival rates assume that some people will die of other causes and compare the observed survival with that expected for people without the cancer. This is a better way to see the impact of the cancer on survival.

To get 5-year survival rates, doctors have to look at people who were treated at least 5 years ago. Improvements in treatment since then may result in a more favorable outlook for people diagnosed with bone cancer today.

Survival rates are often based on previous outcomes of large numbers of people who had the disease, but they cannot predict what will happen in any particular person’s case. Many factors may affect a person’s outlook, such as the type and grade of the cancer, the patient’s age, where the cancer is located, the size of the tumor, and the treatment received. Your doctor can tell you how the numbers below may apply to you, as he or she is familiar with the aspects of your situation.

For all cases of bone cancer combined (in both adults and children), the 5-year relative survival is about 70%. For adults, the most common bone cancer is chondrosarcoma, which has a 5-year relative survival of about 80%. (Survival statistics for Ewing tumors and osteosarcoma can be found in our information about those cancers.)

Research on bone cancer is now being done at many medical centers, university hospitals, and other institutions around the world. There are many clinical trials focusing on bone cancer.

Because primary bone cancer is rare in adults, it's been hard to study well. Most experts agree that treatment in a clinical trial should be considered, especially for people with advanced bone cancers (those that come back after treatment, don't respond to treatment, and/or spread to other parts of the body). This way people can get the best treatment available now and may also get the treatments that are thought to be even better.

Treatment

Some clinical trials are looking into ways to combine surgery, radiation therapy, and chemotherapy (chemo), and drugs known as targeted therapy to treat these cancers.

Chemotherapy

Some studies are testing new chemo drugs. Researchers are also looking for new, and maybe better, ways to use the drugs we have. For instance, doctors are studying whether adding a bisphosphonate called zoledronic acid (Zometa) to the bone cement used to fill in the space left after removing a giant cell tumor might decrease the chance that the tumor will come back in that place.

Another area of interest is long-term chemotherapy side effects. Bone cancers are some of the more common cancers in young people, and doctors are trying to learn more about how the chemo drugs used might affect the way the brain develops and works as survivors grow older.

Targeted therapy

Targeted therapy drugs work differently from standard chemo. These drugs target certain genes and proteins in cancer cells.

A huge area of primary bone cancer research is learning more about the genetic changes in these cancer cells. Researchers are using existing drugs and developing new targeted drugs that focus on these gene changes. It's hoped that these drugs can change the cancer's ability to grow and spread, providing a new and better way to treat these tumors.

For instance, researchers have found that some giant cell tumors that have low levels of certain genes (called microRNA genes) are able to grow and spread faster. MicroRNA changes have also been found in chondrosarcomas. Tests that find these microRNA changes may be helpful in diagnosing these tumors. Finding drugs that target these genes might also prove to be a possible treatment.

One targeted therapy drug, nivolumab (Opdivo), is already used to treat other kinds of cancer. Doctors are trying to find out the best dose to use. They are looking at whether combining it with other treatments might slow tumor growth and help people with advanced sarcomas live longer.

The targeted therapy drug dasatinib (Sprycel) is also used to treat other cancers. Early studies have suggested it may help treat chondrosarcomas, both alone and combined with chemo. Chondrosarcomas seldom respond to chemo or radiation, so targeted therapy drugs may lead to new treatments for these hard-to-treat cancers.

Denosumab (Prolia or Xgeva) is another targeted therapy being tested to see if it can help control giant cell tumor of the bone and/or keep it from coming back after treatment. It's also been shown to help keep giant cell tumors from coming back after surgery.

Overall, results of targeted therapy research are not yet clear. More research is needed in this area, and many clinical trials are testing these treatments.

Radiation

The most common type of radiation used to treat cancer uses beams of x-rays. Doctors are looking for better types of radiation. Proton beam radiation uses particles made up of protons. (Protons are small positively charged particles that are part of atoms.) Proton radiation is already used to treat bone tumors near very sensitive organs, like the brain or the spine. It's being tested on tumors in other parts of the body, too. And as advances make this treatment even more precise and more widely available, it may be found to work better in treating bone tumors.

Another much less common form of particle radiation that has been used to treat chordomas and chondrosarcomas is carbon ion radiation. This may be helpful in treating tumors that do not respond to available treatments, but a lot more research is needed. This treatment is only available in 10 centers worldwide, and there are no carbon ion radiation facilities in North America as of 2017.

Genetics

In addition to clinical trials, researchers are making progress in learning about the causes of bone tumors. For example, changes to the T gene have been found in a few families where more than one member has a chordoma. This might help doctors find specific gene changes that might put a person at higher risk for this type of bone cancer. 

Other gene changes found in giant cell tumors may help doctors find better ways to both diagnose and treat these tumors.

Scientists hope that learning more about the DNA changes that cause bone cancers will also lead to better treatments that might be aimed at these gene defects.

Pain

Pain in the affected bone is the most common sign of bone cancer. At first, the pain is not constant. It may be worse at night or when the bone is used, for instance, leg pain when walking. As the cancer grows, the pain will be there all the time, and get worse with activity.

Swelling

Swelling in the area of the pain may not occur until weeks later. It might be possible to feel a lump or mass depending on where the tumor is.

Cancers in the bones of the neck can cause a lump in the back of the throat that can lead to trouble swallowing or make it hard to breathe.

Fractures

Bone cancer can weaken the bone it's in, but most of the time the bones do not fracture (break). People with a fracture next to or through a bone tumor usually describe sudden severe pain in a bone that had been sore for a few months.

Other symptoms

Cancer in the bones of the spine can press on nerves, causing numbness and tingling or even weakness.

Cancer can cause weight loss and fatigue. If the cancer spreads to internal organs it may cause other symptoms, too. For instance, if the cancer spreads to the lungs, it can cause trouble breathing.

These symptoms are more often due to conditions other than cancer, such as injuries or arthritis. Still, if these problems go on for a long time without a known reason, you should see your doctor.

Surgery is the primary (main) treatment for most kinds of bone cancer. Surgery may also be needed to do a biopsy of the cancer (take out some of the tumor so it can be tested in the lab). The biopsy and the surgical treatment are separate operations, but it's very important that the doctor plans both together. It's best if the same surgeon does both the biopsy and the main surgery. A biopsy taken from the wrong place can lead to problems when the surgeon does the operation to remove the cancer. Sometimes a poorly done biopsy can make it impossible to remove the cancer without cutting off the limb.

The main goal of surgery is to remove all of the cancer. If even a few cancer cells are left behind, they can grow and make a new tumor. To try to be sure that this doesn’t happen, surgeons remove the tumor plus some of the normal tissue around it. This is called wide-excision. Taking out some normal tissue helps ensure that all of the cancer is removed.

After surgery, a pathologist will look at the tissue that was removed to see if the margins (outer edges) have cancer cells. If cancer cells are seen at the edges of the tissue, the margins are called positive. Positive margins can mean that some cancer was left behind. When no cancer is seen at the edges of the tissue, the margins are said to be negative, clean, or clear. A wide-excision with clean margins minimizes the risk that the cancer will grow back where it started.

Tumors in the arms or legs

Sometimes the entire limb needs to be removed in order to do a good wide-excision and remove all of the cancer. This operation is called an amputation. But most of the time the surgeon can remove the cancer without amputation. This is called limb-salvage or limb-sparing surgery.

When taking about treatment options, it's important to discuss the advantages and disadvantages with either type of surgery. For example, most people prefer limb-salvage over amputation, but it's a more complex operation and can have more complications. Both operations have the same overall survival rates when done by expert surgeons. Studies looking at quality of life have shown little difference in how people react to the final result of the different procedures. Still, emotional issues can be very important and support and encouragement are needed for all patients.

No matter which type of surgery is done, rehabilitation will be needed afterward. This can be the hardest part of treatment. If possible, the patient should meet with a rehab specialist before surgery to understand what will be involved.

Amputation

Amputation is surgery to remove part or all of a limb (an arm or leg). When used to treat cancer, amputation removes the part of the limb with the tumor, some healthy tissue above it, and everything below it. In the past, amputation was the main way to treat bone cancers in the arms or legs. Now, this operation is only needed if there's a reason not to do limb-salvage surgery. For example, an amputation may be needed if removing all of the cancer also means removing key nerves, arteries, or muscles that would leave the limb without good function.

MRI scans and examination of the tissue by the pathologist at the time of surgery can help the surgeon decide how much of the arm or leg needs to be removed. Surgery is planned so that muscles and the skin will form a cuff around the amputated bone. This cuff fits into the end of an artificial limb (or prosthesis). After surgery, a person must learn how to use the prosthesis in rehabilitation. With proper physical therapy, people are often walking again 3 to 6 months after leg amputation.

Limb-salvage surgery

The goal of limb-salvage surgery is to remove all of the cancer and still leave a working leg or arm. Most patients with bone cancer in a limb are able to have their limb spared. This type of surgery is very complex and requires surgeons with special skills and experience. The challenge for the surgeon is to remove the entire tumor while still saving the nearby tendons, nerves, and vessels. This is not always possible. If a cancer has grown into these structures, they will need to be removed along with the tumor. This can sometimes result in a limb that's painful or can’t be used. In that case, amputation may be the best option.

In limb-salvage surgery, a wide-excision is done to remove the tumor. A bone graft or an endoprosthesis ( meaning internal prosthesis) is used to replace the bone that's lost. Endoprostheses are made of metal and other materials. Some used in growing children can be made longer without any extra surgery as the child grows.

Further surgery could be needed if the bone graft or endoprosthesis becomes infected, loose, or broken. Limb-salvage surgery patients may need more surgery over the next 5 years, and at some point may need an amputation.

Rehab is much more intense after limb-salvage surgery than it is after amputation. It takes about a year for patients to learn to walk again after limb-salvage of a leg. If the patient does not take part in the rehabilitation program, the salvaged arm or leg could become useless.

Reconstructive surgery

After amputation, surgery can be done to rebuild or reconstruct a new limb. For instance, if the leg must be amputated mid-thigh, the lower leg and foot can be rotated and attached to the thigh bone. The old ankle joint then becomes the new knee joint. This surgery is called rotationplasty. A prosthesis is used to make the new leg the same length as the other (healthy) leg.

If the bone tumor is located in the upper arm, the tumor may be removed and then the lower arm attached again. This leaves the patient with an arm that works but is much shorter.

Tumors in other areas

Bone cancer in the pelvis is treated with a wide-excision when possible. If needed, bone grafts can be used to rebuild the pelvic bones.

For a tumor in the lower jaw bone, the entire lower half of the jaw may be removed and later replaced with bones from other parts of the body.

For tumors in areas like the spine or the skull, it may not be possible to safely do a wide-excision. Cancers in these bones may require a combination of treatments such as curettage, cryosurgery, and radiation.

Curettage

In curettage, the doctor scrapes out the tumor without removing a section of the bone. This leaves a hole in the bone. In some cases, after most of the tumor has been removed, the surgeon will treat the nearby bone tissue to kill any remaining tumor cells. This can be done with cryosurgery or by using bone cement.

Cryosurgery

For this treatment, liquid nitrogen is poured into the hole that's left in the bone after the tumor was removed. This extreme cold kills tumor cells by freezing them. This treatment is also called cryotherapy. After cryosurgery, the hole in the bone can be filled by bone grafts or with bone cement.

Bone cement

The bone cement PMMA (polymethylmethyacrylate) starts out as a liquid and hardens over time. It's put into the hole in the bone in liquid form. As it hardens, it gives off a lot of heat. The heat helps kill any remaining tumor cells. This allows PMMA to be used without cryosurgery for some types of bone tumors.

Surgical treatment of metastasis

To be able to cure a bone cancer, it and any existing metastases must be removed completely with surgery. The lungs are the most common site of distant spread for bone cancer. Surgery to remove bone cancer metastases to the lungs must be planned very carefully. Before the operation, the surgeon will consider the number of tumors, where they are (in one lung or both lungs), their size, and the person’s overall health.

The chest CT scan might not show all the tumors. The surgeon will have a treatment plan ready just in case more tumors are found during the operation than can be seen on the chest CT scan.

Removing all the lung metastases is probably the only chance for a cure. Still, not all lung metastases can be removed. Some tumors are too big or are too close to important structures in the chest (such as large blood vessels) to be removed safely. People whose general health is not good (due to poor nutritional status or problems with the heart, liver, or kidneys) may not be able to deal with the stress of anesthesia and surgery to remove metastases.

For more information, see Cancer Surgery.

Chemotherapy (chemo) is the use of drugs to treat cancer. Chemo is systemic treatment. This means that the drugs go into the bloodstream and circulate to reach and destroy cancer cells all over the body.

Chemo is often a part of treatment for Ewing sarcoma and osteosarcoma.

It isn’t used often for other bone cancers, like giant cell tumors, chordomas, and chondrosarcomas. These types aren’t very sensitive to chemo, so it doesn’t work well. It can be useful for a certain type of chondrosarcoma called mesenchymal and high-grade dedifferentiated chordomas. It may be used along with targeted therapy for some giant cell tumors.

Chemo is sometimes used for bone cancer that has spread through the bloodstream to the lungs and/or other organs.

Commonly used chemo drugs

The drugs mainly used to treat bone cancer include:

• Doxorubicin (Adriamycin^®)
• Cisplatin
• Etoposide (VP-16)
• Ifosfamide (Ifex^®)
• Cyclophosphamide (Cytoxan^®)
• Methotrexate
• Vincristine (Oncovin^®)

In most cases, several drugs (2 or 3) are given together.

Side effects of chemotherapy

Chemo kills cancer cells, but it also damages some normal cells. You will be closely watched during treatment and your team will try to prevent or limit side effects. The side effects of chemo depend on the type of drugs, the doses used, and the length of time they're taken.

Some common short-term side effects include:

• Nausea and vomiting
• Loss of appetite
• Hair loss
• Mouth sores

It's important to tell your cancer care team about any side effects you have so they can be treated.

Chemotherapy can damage the blood-producing cells in the bone marrow, so you may have low blood cell counts. Low blood cell counts can result in:

• Increased chance of infection (too few white blood cells)
• Easy bleeding or bruising after minor cuts or injuries (too few platelets)
• Fatigue or shortness of breath (too few red blood cells)

While you're getting chemo, your doctor will order lab tests to be sure your blood cell counts are at safe levels.

Some side effects are linked to certain drugs. For example:

• Ifosfamide and cyclophosphamide can damage the lining of the bladder and cause bloody urine. This is called hemorrhagic cystitis. It can be prevented by giving a drug called mesna along with the chemo.
• Cisplatin may cause nerve damage (called peripheral neuropathy) leading to problems with numbness, tingling, and even pain in the hands and feet. Kidney damage (called nephropathy) can also occur after treatment with cisplatin. Giving lots of fluid before and after the drug is infused can help prevent this. Cisplatin can sometimes cause problems with hearing (known as ototoxicity). Most often patients with this problem notice they have trouble hearing high-pitched sounds. Your doctor may have you get a hearing test (called an audiogram) before giving cisplatin.
• Over time, doxorubicin can damage the heart. The risk of this goes up as the total amount of the drug given goes up. Before giving doxorubicin, your doctor may test your heart function to make sure that it's safe to give you this drug.

It’s important to note that many of the serious side effects are rare, but they can happen. Talk with your cancer care team so you know what to expect from the chemo you're getting.

The doctors and nurses will watch closely for side effects. There are treatments for most side effects, but preventing them is important. Most side effects, if not all of them, will go away over time after treatment is over. Do not hesitate to ask your cancer care team any questions about side effects.

To learn more, see the chemotherapy section of our website.

The American Cancer Society’s estimates for cancer of the bones and joints for 2018 are:

• About 3,450 new cases will be diagnosed
• About 1,590 deaths from these cancers are expected

Primary cancers of bones account for less than 0.2% of all cancers.

In adults, over 40% of primary bone cancers are chondrosarcomas. This is followed by osteosarcomas (28%), chordomas (10%), Ewing tumors (8%), and malignant fibrous histiocytoma/fibrosarcomas (4%). The remainder of cases are several rare types of bone cancers.

In children and teenagers (those younger than 20 years), osteosarcoma (56%) and Ewing tumors (34%) are much more common than chondrosarcoma (6%).

Chondrosarcomas develop most often in adults, with an average age at diagnosis of 51. Less than 5% of cases occur in patients younger than 20.

Chordomas are also more common in adults. Less than 5% of cases occur in patients younger than 20.

Both osteosarcomas and Ewing tumors occur most often in children and teens.

Visit the American Cancer Society’s Cancer Statistics Center for more key statistics.

A risk factor is anything that affects your chance of getting a disease such as cancer. Different cancers have different risk factors. For example, exposing skin to strong sunlight is a risk factor for skin cancer. Smoking is a risk factor for cancers of the lung, mouth, larynx, bladder, kidney, and several other organs. But having a risk factor, or even several, does not mean that you will get the disease. Most people with bone cancers do not have any apparent risk factors.

Genetic disorders

A very small number of bone cancers (especially osteosarcomas) appear to be hereditary and are caused by defects (mutations) in certain genes. Retinoblastoma is a rare eye cancer in children that can be hereditary. The inherited form of retinoblastoma is caused by a mutation (abnormal copy) of the RB1 gene. Those with this mutation also have an increased risk of developing bone or soft tissue sarcomas. Also, if radiation therapy is used to treat the retinoblastoma, the risk of osteosarcoma in the bones around the eye is even higher.

Finally, there are families with several members who have developed osteosarcoma without inherited changes in any of the known genes. The gene defects that may cause cancers in these families haven’t been discovered yet.

Chondrosarcomas

Multiple exostoses (sometimes called multiple osteochondromas) syndrome is an inherited condition that causes many bumps on a person’s bones. These bumps are made mostly of cartilage. They can be painful and deform and/or fracture bones. This disorder is caused by a mutation in any one of the 3 genes EXT1, EXT2, or EXT3. Patients with this condition have an increased risk of chondrosarcoma.

An enchondroma is a benign cartilage tumor that grows into the bone. People who get many of these tumors have a condition called multiple enchondromatosis. They have an increased risk of developing chondrosarcomas.

Chordomas

Chordomas seem to run in some families. The genes responsible have not yet been found, but familial chordoma has been linked to changes on chromosome 7.

Patients with the inherited syndrome tuberous sclerosis, which can be caused by defects (mutations) in either of the genes TSC1 and TSC2, seem to have a high risk of chordomas during childhood.

Paget disease

Paget disease is a benign (non-cancerous) but pre-cancerous condition that affects one or more bones. It results in formation of abnormal bone tissue and occurs mostly in people older than 50. Affected bones are heavy, thick, and brittle. They are weaker than normal bones and more likely to fracture (break). Most of the time, Paget disease is not life threatening. Bone cancer (usually osteosarcoma) develops in about 1% of those with Paget disease, usually when many bones are affected.

Radiation

Bones that have been exposed to ionizing radiation may also have a higher risk of developing bone cancer. A typical x-ray of a bone is not dangerous, but exposure to large doses of radiation does pose a risk. For example, radiation therapy to treat cancer can cause a new cancer to develop in one of the bones in the treatment area. Being treated when you are younger and/or being treated with higher doses of radiation (usually over 60 Gy) increases your risk of developing bone cancer.

Exposure to radioactive materials such as radium and strontium can also cause bone cancer because these minerals build up in bones.

Non-ionizing radiation, like microwaves, electromagnetic fields from power lines, cellular phones, and household appliances, does not increase bone cancer risk.

Bone marrow transplantation

Osteosarcoma has been reported in a few patients who have undergone bone marrow (stem cell) transplantation.

Injuries

People have wondered if injury to a bone can cause cancer. This has never been proven. Many people with bone cancer remember having hurt that part of their bone. Most doctors believe that these injuries did not cause the cancer. Instead, the cancer caused people to remember the incident or that the injury drew their attention to that bone, making them notice a problem that had already been present for some time.

The exact cause of most bone cancers is not known. Still, scientists have found that bone cancers are linked to a number of other conditions, which are described in the section on risk factors. Still, most people with bone cancers do not have any known risk factors. Research is underway to learn more about the causes of these cancers.

Scientists have made great progress in understanding how certain changes in a person’s DNA can cause normal cells to become cancer. DNA carries the instructions for nearly everything our cells do. We usually look like our parents because they are the source of our DNA. But DNA affects more than just the way we look. It may influence our risks for developing certain diseases, including some kinds of cancer.

DNA is divided into units called genes. Genes carry the recipes for making proteins, the molecules that determine all cell functions. Some genes contain instructions to control when our cells grow and divide.

• Genes that promote cell division are called oncogenes
• Genes that slow down cell division or make cells die at the right time are called tumor suppressor genes

Cancers can be caused by DNA mutations (defects) that activate oncogenes or inactivate tumor suppressor genes. Some people with cancer have DNA mutations that they inherited from a parent. These mutations increase their risk for the disease.

The DNA mutations that cause some inherited forms of bone cancers are known. (See Risk Factors for Bone Cancer) In many cases, genetic testing can be used to see if someone has one of these mutations.

Most bone cancers are not caused by inherited DNA mutations. They're the result of mutations during the person’s lifetime. These mutations may result from exposure to radiation or cancer-causing chemicals, but most often they occur for no apparent reason. These mutations are present only in the cancer cells, so they cannot be passed on to the person’s children.

Scientists are making progress in understanding this process, but there are still some points that are not completely understood. As they learn more, they hope to find ways to better prevent and treat bone cancers.

There are tests that can be used to find some cancers early (such as breast, cervical, colorectal, and skin), before they cause symptoms. At this time, no special tests are available to find bone cancers early. The best way to find these cancers early is to see a health care provider right away if you have signs and symptoms of this disease.

As you cope with cancer and cancer treatment, you need to have honest, open discussions with your doctor. You should be able to ask any question no matter how small it might seem. Nurses, social workers, and other members of the treatment team may also be able to answer many of your questions.

• What kind of bone cancer do I have?
• Has my cancer spread?
• What is the stage of my cancer and what does that mean?
• What are my treatment choices?
• What do you recommend and why?
• What risks or side effects are there to the treatments you suggest?
• What are the chances of my cancer coming back with these treatment plans?
• What should I do to be ready for treatment?
• Based on what you’ve learned about my cancer, how long do you think I’ll survive?

In addition to these sample questions, be sure to write down some of your own. For instance, you might want more information about recovery times so that you can plan your work schedule. Or you might want to ask about second opinions or about clinical trials. You can find more information about communicating with your health care team in The Doctor-Patient Relationship.

For some people with bone cancer, treatment may remove or destroy the cancer. Completing treatment can be both stressful and exciting. You may be relieved to finish treatment, but find it's hard not to worry about cancer coming back. This is very common if you've had cancer.

For other people, the cancer might never go away completely. Some people may get regular treatment with chemotherapy or targeted therapy or other treatments to try and help keep the cancer in check. Learning to live with cancer that does not go away can be difficult and very stressful. 

Life after bone cancer means returning to some familiar things and also making some new choices.

Follow-up care

When treatment ends, your doctors will still want to watch you closely. It's very important to go to all of your follow-up appointments. During these visits, your doctors will ask about any problems you might be having. Exams, lab tests, x-rays, and scans will be needed to look for signs that the cancer has come back. These may be done every 3 to 6 months for a few years. You'll see your doctor and have imaging scans quite often at first, but as time goes on there will be more time between scans and visits. Because primary bone tumors tend to come back, you may need to have imaging scans every year for many, many years.

Your doctor will also look for treatment side effects. Almost every cancer treatment has side effects. Some may last for a few weeks to months, but others can last the rest of your life. Now is the time for you to talk to your cancer care team about any changes or problems you notice and any questions or concerns you have.

After bone surgery, fitting for a prosthetic limb, rehabilitation, and/or physical therapy might be important to help you regain as much of your mobility and independence as possible.