Bone cancer (sarcoma)

Page last reviewed: 13/07/2011

Primary bone cancer is cancer that starts in the bone, rather than starting in another part of the body and then spreading to the bone. The latter is known as secondary bone cancer. Unlike primary bone cancer, secondary bone cancer is common in cases of advanced cancer.

A painful feeling in your bone is the most common symptom of primary bone cancer.

How common is bone cancer?

Primary bone cancer is a rare type of cancer that accounts for just one in every 500 cases of cancer in Ireland each year. There are an estimated 36 cases every year. The cause of most cases of bone cancer is unknown.

Types of primary bone cancer

The four most common types of primary bone cancer are:

  • osteosarcoma
  • Ewing's sarcoma
  • chondrosarcoma
  • spindle cell sarcoma

The different types are described below.

Osteosarcoma

Osteosarcoma is the most common type of bone cancer. Most cases of osteosarcoma develop in children and young people who are between the ages of five and 20, making it the third most common cancer in young people (after leukaemia and brain tumours).

Osteosarcoma usually develops in the larger bones, such as the thigh bone (femur) or the shin bone (tibia).

Ewing's sarcoma

As with osteosarcoma, Ewing's sarcoma usually develops in children and young people aged 10-20, although 10% of cases develop in people who are over 20.

Ewing's sarcoma usually develops in the pelvis, thigh bone or shin bone.

Chondrosarcoma

This type of bone cancer usually develops in adults who are between the ages of 40 and 50.

Chondrosarcoma begins in the cartilage cells before spreading to the bone. Cartilage is a tough, flexible tissue that lines the surface of many bones and joints.

The most common sites for chondrosarcoma to develop are:

  • pelvis
  • thigh bone
  • upper arm bone (humerus)
  • shoulder blade (scapula)
  • ribs

Spindle cell sarcoma

Spindle cell sarcoma is very similar to osterosarcoma in terms of its symptoms and treatment, but it affects older adults (who are 40 or over).

Rarer types of bone cancer

Rarer types of bone cancer include:

  • chordoma, a type of cancer that starts in the base of the spine and usually affects men between the ages of 40 and 50
  • angiosarcomas, a type of cancer than can start anywhere in the skeleton (sometimes in multiple sites at once) and usually affects adults who are aged 20 or over

Outlook

The most important factor in determining the likely outlook for cases of bone cancer is whether the cancer has spread from the bone to other parts of the body (metastasis).

The most common places in the body for the cancer to spread to are the lungs, and secondary lung cancer can be challenging to treat.

If bone cancer is diagnosed before it spreads out of the bone(s), the outlook is moderate to good because a cure is often achievable. This type of cancer is known as localised bone cancer.

Health professionals use a general measurement of a "five-year survival rate" when describing cancer statistics. However, it's important to make clear that the five-year measurement is not an absolute measurement and it does not mean that people with bone cancer only have a five-year life expectancy.

For cases of localised bone cancer:

  • Between 55%-70% of people with osteosarcoma will live for at least five years after receiving the diagnosis.
  • About 70% of people with Ewing's sarcoma will live for at least five years after receiving the diagnosis.
  • About 80% of people with chondrosarcoma will live for at least five years after receiving the diagnosis.
  • About 60% of people with spindle cell sarcoma will live for at least five years after receiving the diagnosis.

If the bone cancer has spread to other parts of the body, the outlook is not as favourable because a cure is often not possible. This type of bone cancer is known as metastatic bone cancer.

For cases of metastatic bone cancer:

  • About 30% of people with osteosarcoma will live for at least five years after receiving a diagnosis.
  • About 30% of people with Ewing's sarcoma will live for at least five years after receiving a diagnosis.
  • Between 10%-30% of people with chondrosarcoma will live for at least five years after receiving a diagnosis.
  • About 25% of people with spindle cell sarcoma will live for at least five years after receiving a diagnosis.

Bone cancer is usually treated with a combination of chemotherapy, radiotherapy, and surgery.

Blood
Blood supplies oxygen to the body and removes carbon dioxide. It is pumped around the body by the heart.
Bone marrow
Bone marrow is the soft, spongy tissue in the centre of bones that produces blood cells.
Joint
Joints are the connection point between two bones that allow movement.
Malignant
Malignant is a term used to describe a life-threatening or worsening condition. In the case of tumours, malignant means cancerous.
Prognosis
A prognosis is a prediction of the possible outcome of a disease or condition.
Spine
The spine supports the skeleton, and surrounds and protects the delicate spinal cord and nerves. It is made up of 33 bones called the vertebrae.
Tissues
Body tissue is made up of groups of cells that perform a specific job, such as protecting the body against infection, producing movement or storing fat.

Page last reviewed: 13/07/2011

Bone pain is the most common symptom of bone cancer. It usually begins with a feeling of tenderness in the affected bone, and then gradually progresses to a persistent ache that often feels worse during the night and when using the affected bone.

In cases of Ewing's sarcoma, bone pain is a symptom that usually gets stronger quite quickly.

Bone pain that is caused by bone cancer is sometimes wrongly mistaken for arthritis in adults and "growing pains" in children and teenagers.

If you have bone cancer, you may also experience inflammation (swelling) or detect a noticeable lump on or around the affected bone. If the bone is near a joint, the swelling may make using the joint difficult.

Less common symptoms of bone cancer include:

  • a high temperature (fever) of 38C (100.4F) or above
  • unexplained weight loss
  • sweating

When to seek medical advice

If your child is experiencing persistent bone pain that lasts for more than three days, take them to visit your GP. Bone pain is unlikely to be the result of growing pains if it lasts longer than three days.

You should also visit your GP if you are an adult and you experience persistent bone pain. While the majority of cases of bone pain are caused by arthritis, the symptom requires a medical diagnosis. If you have arthritis, you will also benefit from having an early diagnosis.

Joint
Joints are the connection point between two bones that allow movement.
Lungs
Lungs are a pair of organs in the chest that control breathing. They remove carbon dioxide from the blood and replace it with oxygen.
Numbness
Numbness refers to a lack of sensation in a part of the body.
Pain
Pain is an unpleasant physical or emotional feeling that your body produces as a warning sign that it has been damaged.

Page last reviewed: 13/07/2011

How does cancer begin?

Cancer begins with an alteration to the structure of the deoxyribonucleic acid (DNA) that is found in all human cells. This is known as a genetic mutation. The DNA provides the cells with a basic set of instructions, such as when to grow and reproduce.

The mutation changes these instructions so that the cells carry on growing. This causes them to reproduce uncontrollably, producing a lump of tissue known as a tumour.

How does cancer spread?

Most cancers grow and spread to other parts of the body via the lymphatic system.

The lymphatic system is a series of glands (or nodes) that are spread throughout your body, much like your blood circulation system. The lymph glands produce many of the specialised cells that are needed by your immune system.

Bone cancer is unusual in that as well as spreading via the lymphatic system it can also spread via the blood. This is why it often spreads to the lungs as cancerous cells can "leak" out of the bone into the blood and then travel into the lungs.

Known risk factors

Bone cancer is a poorly understood condition and, as a result, only a small number of risk factors for the condition have been identified. These are outlined below.

  • Previous exposure to high doses of radiation, such as undergoing radiotherapy; however, the increased risk that is associated with radiotherapy is small.
  • Having Paget's disease, which is an uncommon bone condition that causes weakening of the bones. Around one in 100 people with the condition will develop bone cancer in later life.
  • Having a rare genetic condition known as Li-Fraumeni syndrome.
  • Having a previous history of retinoblastoma, which is a rare type of childhood cancer that develops in the eye.

Research has also found that babies who are born with an umbilical hernia are three times more likely to develop Ewing sarcoma than the population at large.

An umbilical hernia occurs in 1%-2% of all births and is where tissue pokes through the part of the abdomen near to the navel (belly button). It is thought that as an unborn baby develops, the unknown factors that cause the hernia to develop may also contribute to an increased risk of Ewing's sarcoma.

However, the increased risk is still very small in relative terms, as only one in 110,000 children with an umbilical hernia will go on to develop Ewing's sarcoma.

Possible risk factors

A possible risk factor for osterosarcoma is experiencing rapid bone growth during puberty (a growth spurt). Most teenagers who develop osterosarcoma are taller than average.

The possible link between growth spurts and osterosarcoma may also explain why most cases develop in teenagers.

One study found a possible increased risk of osterosarcoma in people whose parents worked in farming around the time they were conceived. This may be due to exposure to pesticides.

However, the researchers who carried out the study emphasised that it was a small study and research involving a larger number of people is needed to confirm or deny this suggested risk factor.

Benign
Benign refers to a condition that should not become life-threatening. In relation to tumours, benign means not cancerous.
Doses
Dose is a measured quantity of a medicine to be taken at any one time, such as a specified amount of medication.
Gene
Genes contain information that you inherit from your parents, such as eye or hair colour. They are carried by chromosomes.
Genetic
Genetic is a term that refers to genes (the characteristics inherited from a family member).

Page last reviewed: 13/07/2011

If you visit your GP with symptoms of bone pain, they will probably carry out a physical examination of the affected bone. They may also refer you for blood tests, which can sometimes be useful in confirming or ruling out other conditions such as an infection or some types of arthritis.

If no obvious cause can be found, it is likely that you will be referred to an orthopaedic surgeon (a specialist in bone conditions) at your local hospital for further testing.

X-rays

The surgeon may arrange a series of X-rays of your affected bone to be taken.

X-rays can often detect damage to the bones that is caused by cancer, or new bone cells that have started to grow around the cancer. They are also often useful in determining whether you have primary or secondary bone cancer.

X-rays cannot provide a definitive diagnosis of bone cancer, but they can indicate whether further investigation is needed.

If your X-rays suggest that you may have bone cancer, it is likely that you will be referred to a specialist centre with expertise in diagnosing and treating the condition.

As bone cancer is very rare, it makes sense to have a small number of specialist centres, rather than spreading resources and trained staff across the whole of the health service.

MRI scan

A magnetic resonance imaging (MRI) scan uses a strong magnetic field and radio waves to produce detailed pictures of the inside of the affected bone.

A MRI scan is an effective way of assessing the size and spread of any cancerous tumour inside the bones.

If you need to have a MRI scan, you will lie on your back and a small "receiving device" will be placed behind, or around, the part of your body being scanned. You will then be moved into a large tube where the scan will take place. The procedure usually lasts about 30 minutes.

A MRI scan is not painful, but some people may find it a little claustrophobic. If you sometimes get claustrophobia, tell the radiographer (who operates the MRI scanner) because they may be able to give you a sedative to help you relax.

CT scan

A computerised tomography (CT) scan involves taking a series of X-rays and using a computer to reassemble them into a detailed three-dimensional (3-D) image of your body.

CT scans are often used to check if the cancer has spread to your lungs. Chest X-rays may also be taken for this purpose.

Bone scans

A bone scan can provide more detailed information about the inside of your bones than an X-ray. A bone scan involves a small amount of radioactive material being injected into your veins. Abnormal areas of bone will absorb the material at a faster rate than normal bone. Therefore, any abnormal areas of bone that are affected by cancer will show up as "hot-spots" on the scan.

Biopsy

The most definitive way of confirming a diagnosis of bone cancer is to take a sample of affected bone and send it to a laboratory for testing. This is known as a biopsy.

A biopsy can also determine exactly what type of bone cancer you have, and what grade your cancer is. A grading system is used to describe how aggressive the cancer is, and how likely it is that it will spread, with high grade cancers being the most aggressive. This information is important for planning treatment.

The two ways that a biopsy can be performed are:

  • A core needle biopsy, performed under a local anesthetic, and is where a long, thin needle is inserted into the bone and used to remove a sample of tissue.
  • An open biopsy, performed under a general anesthetic, and is where the surgeon makes an incision in the affected bone in order to remove a sample of tissue.

You may need to have an open biopsy if the results of a core needle biopsy are inconclusive. 

 

Biopsy
A biopsy is a test that involves taking a small sample of tissue from the body so it can be examined.
MRI
MRI stands for magnetic resonance imaging. It is the use of magnets and radio waves to take detailed pictures of inside the body.
Prognosis
A prognosis is a prediction of the possible outcome of a disease or condition.
Swelling
Inflammation is the body's response to infection, irritation or injury, which causes redness, swelling, pain and sometimes a feeling of heat in the affected area.
Tissue
Body tissue is made up of groups of cells that perform a specific job, such as protecting the body against infection, producing movement or storing fat.  
Veins
Veins are blood vessels that carry blood from the rest of the body back to the heart.
X-ray
An X-ray is a painless way of producing pictures of inside the body using radiation.

Page last reviewed: 13/07/2011

Your care team

If you are diagnosed with bone cancer, it is likely that you will be referred to a specialist centre with experience in treating bone cancer. At specialist bone cancer centres there are teams of specialist health professionals who work together to treat bone cancer. These types of teams are known as multi-disciplinary teams (MDTs).

If you have bone cancer, you may see several, or all, of these healthcare professionals as part of your treatment.

Deciding what treatment is best for you can be difficult. Your cancer team will make recommendations, but the final decision will be yours.

Before going to hospital to discuss your treatment options, you may find it useful to write a list of questions to ask the specialist. For example, you may want to find out what the advantages and disadvantages of particular treatments are.

Your treatment plan

The different types of bone cancer are usually treated in a similar way. Firstly, chemotherapy is given in order to try to shrink the tumour(s). This may then be followed by a course of radiotherapy to shrink the tumour(s) further.

However, in cases of osterosarcoma, radiotherapy is rarely used because it has limited effectiveness in treating this type of bone cancer.

Following chemotherapy and/or radiotherapy, surgery may be carried out to remove the section of cancerous bone. In many cases, it is possible to maintain the function of the body part from where the section of the bone was taken from. This is known as limb-sparing surgery (see below).

In some cases - for example, if the cancer has spread beyond the bone into the surrounding blood vessels - it will be necessary to amputate some, or all, of the limb. However, due to advances in diagnosis and treatment, only a minority of cases (about 15%) require amputation.

If cancer has spread to the lungs, surgery can also be used to remove the affected portion of the lungs. The Health A-Z article about lung cancer provides more information about surgery for lung cancer.

Following surgery, further sessions of radiotherapy and/or chemotherapy may be given in order to destroy any remaining cancerous cells.

Limb-sparing surgery

Limb-sparing surgery is usually possible when the cancer has not spread beyond the bone, and the affected bone itself is in an easily accessible position such as:

  • the arm
  • the leg
  • the shoulder
  • the pelvis
  • the hip

The most common type of limb-sparing surgery involves removing the section of affected bone as well as some of the surrounding tissue (in case any cancerous cells have spread into the tissue). The removed section of bone is then replaced with a metal implant called a prosthesis.

In some cases, it may be possible to take a sample of bone from another part of the body and use it as an alternative to prosthesis. This type of operation is known as a bone graft.

If the cancer is near a joint, such as the knee joint, it may be necessary to remove the joint and replace it with an artificial one, which is made from a combination of plastic, metal, and ceramics.

Artificial joints can be used to replace:

  • a knee joint
  • a hip joint
  • a shoulder joint

The most common complication of limb-sparing surgery is a post-operative infection, which occurs in one out of every 10 cases. Any infection can make the prosthesis or artificial joint unstable. If this occurs, further surgery may be required to repair it.

In the most serious cases of infection, repairing the prosthesis or joint may not be possible and amputation will be required.

Amputation

Amputation is usually required:

  • if the cancer has spread beyond the bone into major blood vessels and/or nerves
  • if the cancer has spread beyond the bone into your skin
  • if you develop a serious infection after limb-sparing surgery
  • if the cancer has developed in a part of the body where limb sparing surgery is not technically possible, such as in the ankle

Being told that you need to have an amputation can be devastating, particularly for children. Your care team will understand the shock and fear that you, or your child, may be feeling and will be able to provide counselling and other support as required.

In some cases, your care team may be able to introduce you or your child to someone who has had an amputation, and they will be able to give you advice and support about living with an amputation.

Artificial limbs are now very advanced and convenient to use. For example, people with an artificial leg are able to walk, run and play sport. In many cases, an artificial limb allows a wider range of movement than a limb that is repaired with limb-sparing surgery.

Research has also found that most people who have an amputation are able to enjoy the same quality of life as people who have limb-sparing surgery.

After having surgery, it is likely that you will be in some pain, particularly around your stump (the point at which your limb was removed) so you will be given painkillers.

Once your stump has healed, you will be fitted with an artificial limb. These are usually custom-made in order to make wearing it and using it as comfortable as possible.

When you are able to leave hospital, you will be referred to a local limb centre. Limb centres provide advice, support and treatment for people with artificial limbs.

At the limb centre, you will be given physiotherapy and occupational therapy (therapy that is designed to improve the skills that you need for day-to-day living) so that you can make the best use of your artificial limb.

Any adjustments to your limb that may be required as you grow older can also be carried out at the limb centre.

Chemotherapy

Chemotherapy uses powerful cancer-killing medication to treat cancer.

There are four ways that chemotherapy can be used to treat bone cancer. It can be used:

  • before surgery to shrink the tumour, making it more likely that you will only require limb-sparing surgery
  • in combination with radiotherapy before surgery (chemoradiation); this approach works particularly well in the treatment of Ewing's sarcoma
  • after surgery in order to prevent the cancer returning
  • to control symptoms in cases where a cure is not possible (this is known as palliative chemotherapy)

Chemotherapy treatments are usually given in cycles. A cycle involves taking the chemotherapy medication for several days, and then having a break for a few weeks to allow your body to recover from the effects of the treatment.

The number of cycles that you require will depend on the type and the grade of your bone cancer. For example, some people may only require six cycles of treatment while others may need up to 14 cycles.

Chemotherapy for bone cancer involves taking a combination of different medications. The medications are usually delivered via a drip into your vein, or into a tube that is connected to one of the blood vessels in your chest.

Side effects of chemotherapy include:

  • nausea
  • vomiting
  • diarrhoea
  • loss of appetite
  • mouth ulcers
  • tiredness
  • skin rashes
  • infertility
  • hair loss (following treatment, your hair should take between three to six months to grow back)

Most of the side effects associated with chemotherapy should resolve once your treatment has finished.

However, there is a risk that you will be permanently infertile. Depending on the type of chemotherapy medication that is used, this risk can range from between 13%-80%. Your care team will be able to provide more detailed information about the specific risk to your fertility.

If you wish to have children, it may be possible to take samples of your sperm (men) or eggs (women) so that they can be used later in fertility treatments, such as IVF.

Chemotherapy can also weaken your immune system, making you more vulnerable to infection. Inform your care team and/or your GP as soon as possible if you experience the possible signs of an infection, such as:

  • a high temperature (fever) of 38C (100.4F) or above
  • coughs
  • sore throat
  • a feeling of being generally unwell

Radiotherapy

Radiotherapy is a type of treatment that uses pulses of radiation to destroy cancerous cells.

As with chemotherapy, radiotherapy can be used before and after surgery to treat bone cancer, or it can be used to control the symptoms and slow the spread of cancer when a cure is not possible.

Radiotherapy is usually given five days a week with a break from treatment over the weekend. Each session of radiotherapy usually lasts around 10-15 minutes. Most people require between two and five weeks of treatment.

Common side effects of radiotherapy include:

  • reddening and irritation of the skin of the body part being treated (this can feel much like sunburn)
  • joint pain in the part of the body that is being treated
  • nausea
  • hair loss in the body part being treated
  • tiredness

These side effects will pass once the radiotherapy has been completed, although feelings of tiredness may persist for several weeks.

 

Chemotherapy
Chemotherapy is a treatment of an illness or disease with a chemical substance, e.g. in the treatment of cancer.
Joint
Joints are the connection point between two bones that allow movement.
Radiotherapy
Radiation therapy uses x-rays to treat disease, especially cancer.
Spine
The spine supports the skeleton, and surrounds and protects the delicate spinal cord and nerves. It is made up of 33 bones called the vertebrae.
Veins
Veins are blood vessels that carry blood from the rest of the body back to the heart.

An multi-disciplinary team is made up of a number of different specialists.

These may include:

  • an orthopaedic surgeon (a surgeon who specialises in bone and joint surgery)
  • a clinical oncologist (a specialist in the non-surgical treatment of cancer)
  • a pathologist (a specialist in diseased tissue)
  • a radiologist (a specialist in radiotherapy)
  • a palliative care specialist (a specialist in pain relief)
  • a social worker
  • a psychologist  
  • a specialist cancer nurse, who will usually be your first point of contact

Page last reviewed: 13/07/2011

Phantom limb pain

Many people who have an amputation find that they experience a sensation that their limb is still attached to their body and is causing them pain. This is known as phantom limb pain.

The causes of phantom limb pain are unknown, but it is thought that the pain may be the result of nerve endings in the stump sending abnormal nerve impulses that your brain interprets as pain.

If, after having a limb amputated, you experience phantom limb pain, let the staff at your limb centre know because there are a number of treatments available, such as:

  • painkillers
  • hypnosis
  • anti-epileptic medications
  • antidepressant medications

Anti-epileptic and antidepressant medications were originally designed to treat epilepsy and depression, but they have since proved to be effective in treating certain types of nerve pain.

Content provided by NHS Choices www.nhs.uk and adapted for Ireland by the Health A-Z.

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