Overview

Primary bone cancer is a rare type of cancer that begins in the bones. Around 550 new cases are diagnosed each year in the UK.

This is a separate condition from secondary bone cancer, which is cancer that spreads to the bones after developing in another part of the body.

Title
Bone cancer

Types

Some of the main types of bone cancer are:

  • Osteosarcoma – the most common type, which mostly affects children and young adults under 20
  • Ewing sarcoma – which most commonly affects people aged between 10 and 20
  • Chondrosarcoma – which tends to affect adults aged over 40

Young people can be affected because the rapid growth spurts that occur during puberty may make bone tumours develop.

The above types of bone cancer affect different types of cell. The treatment and outlook will depend on the type of bone cancer you have.

Symptoms

Bone pain is the most common symptom of bone cancer. Some people experience other symptoms as well.

Bone pain

Pain caused by bone cancer usually begins with a feeling of tenderness in the affected bone. This gradually progresses to a persistent ache or an ache that comes and goes, which continues at night and when resting.

Any bone can be affected, although bone cancer most often develops in the long bones of the legs or upper arms.

The pain can sometimes be wrongly mistaken for arthritis in adults and growing pains in children and teenagers.

Other symptoms

Some people also experience swelling and redness (inflammation) or notice a lump on or around the affected bone. If the bone is near a joint, the swelling may make it difficult to use the joint.

In some cases, the cancer can weaken a bone, causing it to break (fracture) easily after a minor injury or fall.

Less common symptoms can include:

Causes

Cancer occurs when the cells in a certain area of your body divide and multiply too rapidly. This produces a lump of tissue known as a tumour.

The exact reason why this happens is often not known, but certain things can increase your chance of developing the condition, including:

These risk factors are described in more detail below.

Radiotherapy treatment

Previous exposure to high doses of radiation during radiotherapy may cause cancerous changes in your bone cells at a later stage, although this risk is thought to be small.

Bone conditions

Some non-cancerous (benign) conditions affecting the bones may increase your chances of developing bone cancer, although the risk is still small.

In particular, a condition called Paget's disease of the bone can increase the risk of bone cancer in people over 50-60 years of age.

Rarer conditions that cause tumours to grow in your bones, such as Ollier's disease, can also increase your risk of bone cancer.

Genetic conditions

A rare genetic condition called Li-Fraumeni syndrome can increase your risk of developing bone cancer, as well as several other types of cancer.

People with this condition have a faulty version of a gene that usually helps stop the growth of tumours in the body.

Other conditions

People who had a rare type of eye cancer called retinoblastoma as a child may be more likely to develop bone cancer, because the same inherited faulty gene can be responsible for both conditions.

Research has also found that babies born with an umbilical hernia are 3 times more likely to develop a type of bone cancer called Ewing sarcoma, although the risk is still very small.

Diagnosis

If you're experiencing bone pain, your GP will ask about your symptoms and examine the affected area, before deciding whether you need to have any further tests.

They will look for any swelling or lumps, and ask if you have problems moving the affected area. They may ask about the type of pain you experience – whether it's constant or comes and goes, and whether anything makes it worse.

After being examined, you may be referred for an X-ray of the affected area to look for any problems in the bones. If the X-ray shows abnormal areas, you'll be referred to an orthopaedic surgeon (a specialist in bone conditions) or bone cancer specialist for a further assessment.

Some of the tests you may have to help diagnose and assess bone cancer are described below.

X-rays

An X-ray is a procedure where radiation is used to produce images of the inside of the body. It's a particularly effective way of looking at bones.

X-rays can often detect damage to the bones caused by cancer, or new bone that's growing because of cancer. They can also determine whether your symptoms are caused by something else, such as a broken bone (fracture).

If an X-ray suggests you may have bone cancer, you should be referred to a specialist centre with expertise in diagnosing and treating the condition. As bone cancer is rare, there are a small number of specialist centres, so you may have to travel outside your local area for advice and treatment.

Biopsy

The most definitive way of diagnosing 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 determine exactly what type of bone cancer you have and what grade it is (see below).

A biopsy can be carried out in two ways:

  • A core needle biopsy is performed under anaesthetic (depending on where the bone is located, this could be a local anaesthetic or general anaesthetic). A thin needle is inserted into the bone and used to remove a sample of tissue.

An open biopsy is performed under a general anaesthetic

  • The surgeon makes an incision in the affected bone to remove a sample of tissue.

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

Further tests

If the results of the biopsy confirm or suggest bone cancer, it's likely you'll have further tests to assess how far the cancer has spread. These tests are described below.

MRI scan

A magnetic resonance imaging (MRI) scan uses a strong magnetic field and radio waves to produce detailed pictures of the bones and soft tissues.

An MRI scan is an effective way of assessing the size and spread of any cancerous tumour in or around the bones.

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 give more detailed information about the inside of your bones than an X-ray. During a bone scan, a small amount of radioactive material is injected into your veins.

Abnormal areas of bone will absorb the material at a faster rate than normal bone and will show up as "hot spots" on the scan.

Bone marrow biopsy

If you have a type of bone cancer called Ewing sarcoma, you may have a test called a bone marrow biopsy to check if the cancer has spread to the bone marrow (the tissue inside your bones).

During the test, a needle is inserted into your bone to remove a sample of your bone marrow. This may be done under either local or general anaesthetic.

Staging and grading

Once these tests have been completed, it should be possible to tell you what stage and grade the bone cancer is. Staging is a description of how far a cancer has spread and grading is a description of how quickly the cancer is likely to spread in the future.

A widely used staging system for bone cancer in the UK uses 3 main stages:

  • Stage 1 – the cancer is low-grade and has not spread beyond the bone
  • Stage 2 – cancer has still not spread beyond the bone, but is a high-grade
  • Stage 3 – cancer has spread into other parts of the body, such as the lungs

Most cases of stage 1 bone cancer and some stage 2 bone cancers have a good chance of being cured. Unfortunately, stage 3 bone cancer is more difficult to cure, although treatment can relieve symptoms and slow the spread of cancer.

Coping with a diagnosis

Being told you have bone cancer can be a distressing and frightening experience. Receiving that type of news can be upsetting at any age, but can be particularly difficult if you are still in your teenage years, or if you're a parent of a child who has just been told they have bone cancer.

These types of feelings can cause considerable stress and anxiety, which in some cases can trigger depression. If you think you may be depressed, your GP may be a good person to talk to about support and possibly treatment.

Treatment

Reatment for bone cancer depends on the type of bone cancer you have, how far it has spread and your general health. The main treatments are surgery, chemotherapy and radiotherapy.

Your treatment plan

Your treatment should be managed by a specialist centre with experience in treating bone cancer, where you'll be cared for by a team of different healthcare professionals known as a multi-disciplinary team (MDT).

Members of the MDT will 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) and a specialist cancer nurse, among others.

Your MDT will recommend what they think is the best treatment for you, but the final decision will be yours.

Your recommended treatment plan may include a combination of:

  • Surgery to remove the section of cancerous bone – it's often possible to reconstruct or replace the bone that's been removed, although amputation is occasionally necessary
  • Chemotherapy – treatment with powerful cancer-killing medication
  • Radiotherapy – where radiation is used to destroy cancerous cells

In some cases, a medication called mifamurtide may be recommended as well.

Surgery

Surgery to remove the cancerous area of bone is an important part of treatment for bone cancer, although it's often combined with the other treatments mentioned below.

It's usually possible to avoid removing an affected body part completely (known as limb-sparing surgery), although up to 1 in every 10 people may need to have a limb permanently removed (amputation).

Limb-sparing surgery

Limb-sparing surgery is usually possible when the cancer has not spread beyond the bone, and the bone can be reconstructed.

The most common type of limb-sparing surgery involves removing the section of affected bone and some of the surrounding tissue (in case any cancerous cells have spread into the tissue).

The removed section of bone may then be replaced with a metal implant called a prosthesis or a piece of bone from elsewhere in your body (bone graft).

If the cancer is near a joint, such as the knee, it may be possible to remove the joint and replace it with an artificial one.

Amputation

Amputation may be necessary if limb-sparing surgery is not possible or has not worked well. For example, it may be necessary if:

  • Cancer has spread beyond the bone into major blood vessels or nerves
  • You developed an infection after limb-sparing surgery and the prosthesis or bone graft had to be removed
  • Cancer has developed in a part of the body where limb-sparing surgery is not technically possible, such as the ankle

Your care team will understand the shock and fear that you, or your child, may be feeling if an amputation is needed and should be able to provide you with counselling and other support. In some cases, your care team may be able to introduce you to someone who has already had an amputation.

After an amputation, most people use an artificial limb to replace the removed limb. These limbs are now very advanced and convenient to use. For example, people with an artificial leg are often able to walk, run and play sport, and have an excellent quality of life.

Recovering from surgery

After limb-sparing surgery or an amputation, you'll need help to return to normal life. This is known as rehabilitation.

Rehabilitation will usually involve physiotherapy sessions, where you carry out exercises to help regain proper function in the treated body part, and occupational therapy, where you're taught skills to help you cope with day-to-day activities.

After an amputation, you may be referred to a local limb centre for advice, support and rehabilitation treatment.

Chemotherapy

There are 4 ways chemotherapy can be used to treat bone cancer:

  • Before surgery – to shrink the tumour and make surgery easier
  • Combination with radiotherapy before surgery (chemoradiation) – this approach works particularly well in the treatment of Ewing sarcoma
  • After surgery, to prevent cancer returning
  • To control symptoms in cases where a cure is not possible (known as palliative chemotherapy)

Chemotherapy for bone cancer involves taking a combination of different medications that are usually delivered via a drip into your vein, or into a line inserted into a bigger blood vessel.

The treatment is usually given in cycles. A cycle involves taking the chemotherapy medication for several days, then having a break for a few weeks to allow your body to recover from the effects of the treatment. The number of cycles you need will depend on the type and grade of your bone cancer.

Side effects

Chemotherapy can damage healthy cells as well as cancerous cells, which means it often causes a number of side effects.

Common side effects of chemotherapy include:

Most side effects associated with chemotherapy should resolve once your treatment has finished. However, there is a risk you will be permanently infertile. Your care team will provide more detailed information about the specific risk to your fertility.

Radiotherapy

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

Radiotherapy for bone cancer involves beams of radiation being directed at the cancerous section of bone by an external machine.

This is normally given in daily sessions, 5 days a week, with each session lasting a few minutes. The whole course of treatment will usually last a few weeks.

Side effects

The radiation you're exposed to during radiotherapy will mostly be focused on the cancerous cells, but healthy cells nearby can also be damaged. This can lead to side effects such as:

  • Reddening and irritation of the skin (this can feel much like sunburn)
  • Joint pain in the part of the body that is being treated
  • Feeling sick
  • 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.

Mifamurtide

For people with a type of bone cancer called osteosarcoma, a medication called mifamurtide may be used alongside the treatments described above.

Mifamurtide is an immune macrophage stimulant. This means it works by encouraging the immune system to produce specialised cells that kill cancerous cells.

It's normally recommended for young people with high-grade osteosarcomas and is given after surgery, in combination with chemotherapy, to help prevent the cancer returning.

Mifamurtide is slowly pumped into one of your veins over the course of an hour (known as infusion). The recommended course of treatment is usually twice a week for 12 weeks, and then once a week for a further 24 weeks.

Side effects

Mifamurtide can cause a wide range of side effects. These can include:

  • Nausea and vomiting
  • Diarrhoea or constipation
  • Headache
  • Dizziness
  • Loss of appetite
  • Tiredness and weakness

It's unclear whether it's safe to take mifamurtide during pregnancy, so as a precaution it's important to use an effective method of contraception if you're a sexually active woman. You will need to tell your MDT as soon as possible if you think you're pregnant, and you should avoid breastfeeding while taking mifamurtide.

Follow-up

Once your treatment has finished, you'll need to attend regular follow-up appointments to check the cancer hasn't returned.

You'll be asked to attend frequent appointments in the first 2 years after treatment has finished – possibly every 3 months. These will become less frequent as the years go on.

Contact your specialist or GP if you develop symptoms of bone cancer again and think the cancer may have come back.