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Bone cancer (sarcoma)

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Bone cancer (sarcoma)



Introduction 

Primary bone cancer is a tumour that starts growing inside a bone.

Cancer that spreads from another part of the body into surrounding bone is known as secondary bone cancer.

The most common symptom of bone cancer is bone pain that usually gets worse over time and can feel more painful during the night.

 

If you or your child is experiencing persistent bone pain that lasts for more than three days, visit your GP. While it is highly unlikely to be the result of bone cancer, it does require further investigation.

 

Types of bone cancer

All types of bone cancer are very rare. The four most common types (although still very rare in general terms) are described below.

Osteosarcoma

Osteosarcoma is the most common type of bone cancer. Most cases develop in teenagers and young adults, but you can get it at any age.

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

Ewing's sarcoma

Ewing's sarcoma is most common in teenagers, although it can also develop in adults.

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

Chondrosarcoma

This type of bone cancer usually develops in adults aged between 30 and 60.

It mostly affects the pelvis, thigh bone, upper arm bone, shoulder blade (scapula) and the ribs.

Spindle cell sarcoma

Spindle cell sarcoma is very similar to osteosarcoma in terms of its symptoms and treatment, but it affects older adults aged 40 or over.

Treating bone cancer

The treatment plan for most cases of bone cancer is to use a course of chemotherapy to shrink the tumour and surgery to remove the affected area of bone.

In the past this often meant that a section of a limb, such as the lower leg, had to be surgically removed (in an amputation), but today it is often possible to retain the limb by replacing the affected bone with a metal implant. This is known as limb-sparing surgery.

 

The outlook for bone cancer depends on whether the cancer has spread from the bone to other parts of the body.

It's easier to cure if the cancer has not spread (known as localised bone cancer). If it has spread (most commonly to the lungs or bone marrow), it can be harder to treat. This is known as metastatic bone cancer.

 

What causes bone cancer?

The reason why a small minority of people develop bone cancer is still unclear.

Known risk factors include:



previous exposure to radiation, such as radiotherapy



a condition known as Paget’s disease of the bone, where the normal cycle of bone growth is disrupted – however, less than 1% of people with Paget’s disease will actually develop bone cancer



 

Coping with cancer


In this video, people who have been through cancer treatment talk about what kept them going and the practicalities of treatment.


Symptoms of bone cancer 

Bone pain is the most common symptom of bone cancer.

It usually begins with a feeling of tenderness in the affected bone, which gradually progresses to a persistent ache that often feels worse during the night and when the bone is in use.

In cases of Ewing's sarcoma, bone pain usually gets worse 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 swelling and redness (inflammation) 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.

In some cases the cancer can weaken a bone so it can become fractured or broken after a minor injury or fall.

Less common symptoms of bone cancer include:



a high temperature (fever) of 38C (100.4F) or above



unexplained weight loss



sweating; usually at night



When to seek medical advice

See your GP if you or your child experiences persistent bone pain that lasts for more than three days. Bone pain is unlikely to be the result of growing pains if it lasts longer than three days and, while it is highly unlikely to be the result of bone cancer, it does require further investigation.

The majority of cases of bone pain in adults are caused by arthritis. If you do have arthritis, you will benefit from having an early diagnosis.

 

Causes of bone cancer 

Cancer begins with a change in the structure of DNA. DNA provides our cells with a basic set of instructions, such as when to grow and reproduce.

A change in DNA structure is known as a mutation, and it can alter the instructions that control cell growth. This means that cells continue to grow instead of stopping when they should. This causes cells to reproduce in an uncontrollable manner, producing a lump of tissue called a tumour.

Known risk factors

Bone cancer is a poorly understood condition and, as a result, only a small number of known risks have been identified. These include:



Fast-growing bones – as the majority of cases of bone cancer develop in teenagers it is thought the rapid growth spurt that occurs during puberty may in some way make bone tissue more vulnerable to cancer. Higher rates of bone cancer have also been found in certain breeds of dogs that experience rapid bone growth.



Previous exposure to high doses of radiation, such as undergoing radiotherapy; however, the increased risk associated with radiotherapy is small.



Paget’s disease of the bone, which is an uncommon bone condition that causes weakening of the bones. However, less than 1 in 100 people with the condition will develop bone cancer in later life.



Rare genetic conditions such as Li-Fraumeni syndrome, which affects around 1 in every 142,000 people.



A history of retinoblastoma. This is a rare type of childhood cancer which develops in the eye. People who have had this condition have an increased risk of developing bone cancer.



Research has also found babies born with an umbilical hernia are three times more likely to develop Ewing’s sarcoma than others.

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

 

How does bone cancer spread?

Left untreated,  cancerous cells can spread from the affected area of bone. This can involve an inward spread into the bone marrow (the jelly-like material found inside bones) or outwards into the blood and then usually into the lungs.

Diagnosing bone cancer 

Your GP will carry out a physical examination of the affected bone. They may also refer you for blood tests to rule out other conditions, such as an infection or some types of arthritis.

Some bone cancers can cause abnormalities in the blood. For example, in some osteosarcomas an increase in an enzyme called alkaline phosphatase can be detected.

You may also be referred for an X-ray which can detect bone cancer (see below). X-rays may also be recommended if you go to an accident and emergency department with a suspected broken bone.

If no obvious cause can be found for your bone pain, you will probably be referred to an orthopaedic surgeon (a specialist in bone conditions) at your local hospital for further tests.

 

X-rays

X-rays can often detect damage to the bones caused by cancer, or new bone that's growing because of cancer.

They can also help determine whether your symptoms are due to another cause, such as a bone fracture.

If your X-rays suggest that 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 many patients have to travel 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 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 will spread, with high-grade cancers being the most aggressive. This information is important for planning treatment.

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 testing

If the results of the biopsy confirm (or at least suggest) bone cancer, you will probably be referred for 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 inside of the affected bone.

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

 

Staging

Once these tests have been completed and the results have come through, it should be possible to tell you what stage and grade the bone cancer is at. 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 in England uses three stages to assess cases of bone cancer:

Stage 1 – the cancer is low-grade and has not spread beyond the bone. Stage 1 can be further divided into two sub-stages:



Stage 1A – the cancer is still limited to the inside of the affected bone.



Stage 1B – the cancer has started to spread into the outer layer of the bone.



Stage 2 – the cancer has still not spread beyond the bone but it is a high-grade type of cancer; again this can be divided into two sub-stages:



Stage 2A – the cancer is still limited to the inside of the affected bone.



Stage 2B – the cancer has started to spread into the outer layer of the bone.



Stage 3  – the cancer has spread into other parts of the body, such as the lungs.

Most cases of stage 1 bone cancer and some of stage 2 bone cancers have a good chance of being cured.

The same is not usually true with stage 3 bone cancer but it may be possible to relieve symptoms and slow the spread of cancer with treatments such as chemotherapy and, in some cases, radiotherapy.

 

Coping with a diagnosis

Being told you have bone cancer can be a distressing, bewildering and frightening experience. Receiving that type of news can be upsetting at any age but particularly cruel if you are still in your teenage years, or if you are 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. You may be depressed if during the past month you have been bothered by feeling down and hopeless and you no longer take pleasure in activities you used to enjoy.

If you think you may be depressed, see your GP as there are a range of effective treatments, such as medication and counselling, that can help.

 

You may find it useful to visit the Bone Cancer Research Trust website, which is the UK’s leading charity for people affected by bone cancer.

If you are a teenager you can also contact the Teenage Cancer Trust, a charity for teenagers and young adults affected by cancer.

 

Treatment for bone cancer 

If you are diagnosed with bone cancer, it's likely you will be referred to a specialist centre with experience in treating bone cancer.

Your care team

At specialist bone cancer centres teams of specialist health professionals work together to treat bone cancer. These teams are known as multi-disciplinary teams (MDTs).

Members of the MDT 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 and symptom control)



a social worker



a psychologist 



a specialist cancer nurse, who will usually be your first point of contact between yourself and the rest of the team



Your treatment plan

Depending on the type of bone cancer you have, the main treatment will include a combination of:



chemotherapy - treatment using powerful cancer-killing medication



radiotherapy - where pulses of radiation are used to destroy cancerous cells



surgery - to remove the section of cancerous bone



In many cases, it is possible to reconstruct the bone that has been removed. This is known as limb-sparing surgery.

However, sometimes the only way to treat bone cancer is to amputate some, or all, of the limb. Due to advances in diagnosis and treatment, only a minority of patients (around one-in-seven cases) need an amputation.

 

Chemotherapy

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



before surgery to shrink the tumour, making it more likely 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 to prevent the cancer returning



to control symptoms in cases where a cure is not possible (known as palliative chemotherapy)



Chemotherapy treatments are 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.

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

Side effects of chemotherapy include:



nausea or vomiting 



diarrhoea



mouth ulcers



tiredness



infertility



hair loss (following treatment, your hair should take between three to six months to grow back)



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.

Chemotherapy can also weaken your immune system, making you more vulnerable to infection.

 

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

Common side effects of radiotherapy include:



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.

 

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 as well as 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. However, there are other ways to replace bone, for example by using 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 may be made from plastic, metal, or ceramics.

 

Amputation

Amputation may be required if you develop a serious complication after limb-sparing surgery, if the cancer involves structures which are important for the function of the limb, or if the cancer:



has spread beyond the bone into major blood vessels or nerves



has spread beyond the bone into your skin



has developed in a part of the body where limb-sparing surgery is not technically possible, such as in the ankle



Being told you need 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 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.

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 repaired with limb-sparing surgery.

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

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. You will also be given physiotherapy and occupational therapy (therapy to improve the skills needed for day-to-day living) to make the best use of your artificial limb.

 

Mifamurtide

A medication called mifamurtide may be used as part of the treatment of high-grade osteosarcoma.

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

Mifamurtide is given after surgery, in combination with chemotherapy, to kill any remaining cancerous cells and help prevent the cancer from returning.  

Mifamurtide is slowly pumped into one of your veins over the course of an hour, this is known as infusion. It is usually carried out at your local hospital (you will be able to go home once the infusion has been completed) or possibly at your GP’s surgery.

The recommended course of treatment is usually twice a week for 12 weeks and then once a week for a further 24 weeks after that.

Mifamurtide can cause a wide range of side effects, usually mild to moderate.

They include:



feeling or being sick



diarrhoea or constipation



headache 



dizziness



loss of appetite



muscle and joint pain



blood in your urine 



blurred vision



hearing loss 



 

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

 

Follow up

If tests show your body is now free of cancerous cells, your treatments will end. But you'll still need to attend regular follow-up appointments to check the cancer hasn't returned.

You will be asked to attend frequent appointments in the first two years after treatment has finished – possibly every two to four months depending on the grade of the cancer. These will become less frequent as the years go on.

 

Outlook

The expected outlook for people with bone cancer is outlined below.

Five-year survival rates

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

 

Osteosarcoma

Six out of 10 people with localised osteosarcoma will live at least five years after diagnosis and most of these people will be completely cured.

The outlook for metastatic osteosarcoma is much poorer as only 1 in 10 people will live  at least five years after being diagnosed.

 

Ewing's sarcoma

Seven out of 10 people with localised Ewing’s sarcoma will live at least five years after diagnosis, and again, most of them will be completely cured.

Only 3 out of 10 people with metastatic Ewing’s sarcoma will live at least five years after diagnosis.

 

Chondrosarcoma

In cases of chondrosarcoma, the grade of the cancer is the most important factor in determining the outlook.

The outlook for low-grade chondrosarcoma is generally good, with 8 out of 10 people living at least five years after a diagnosis.

The outlook for high-grade chondrosarcoma is poorer, with only 3 out of 10 people living  at least five years after diagnosis.

 

Spindle cell sarcoma

Six out of 10 people with localised spindle cell sarcoma will live at least five years after diagnosis.

Only one-in-four people with metastatic spindle cell sarcoma will live at least five years after diagnosis.


 

Weakened bones

In some case, the cancer can severely weaken the affected bone, increasing the risk of a break (fracture). This could be dangerous, as the fracture could then lead to cancerous cells "leaking out" of the bone and moving into surrounding tissue.

If tests show your bone is weakened it may be necessary to protect it by placing it into a splint. A splint is worn to protect a weakened bone while at the same time limiting its range of movement – the less the bone moves, the lower its chances of fracturing.




Bone cancer (sarcoma)