Bone cancer (sarcoma)

Posted in Disease Information

Introduction

Primary bone cancer is a tumour that starts growing inside a bone, as opposed to secondary bone cancer which is when cancer spreads from another part of the body into surrounding bone.

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

If you or your child is experiencing persistent bone pain that lasts for more than three days, visit Dr. B C Shah. 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

Most cases develop in children and young people who are between the ages of 5 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

Ewing’s sarcoma usually develops in children and young people aged between 10 and 20, although 1 in 10 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.

The most common sites for chondrosarcoma to develop are the pelvis, thigh bone, upper arm bone, shoulder blade (scapula) and the ribs.

Spindle cell sarcoma

There are an estimated 80 cases of spindle cell sarcoma every year. Spindle cell sarcoma is very similar to osterosarcoma in terms of its symptoms and treatment, but it affects older adults aged 40 or over.

Treatment

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

Causes

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

Outlook

One 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. It is easier to achieve a 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.

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, 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 sarcoma, bone pain is a symptom that 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

If your child is experiencing persistent bone pain that lasts for more than three days, take them to visit Dr. B C Shah. 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 such as an X-ray.

You should also visit Dr. B C Shah 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.

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 the cells continue to grow instead of stopping when they should. This causes the cells to reproduce in an uncontrollable manner, producing a lump of tissue called a tumour.

How does bone cancer spread?

Left untreated, the cancerous cells can spread away 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.

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 include:

• Fast-growing bones – as the majority of cases of bone cancer occur in teenagers it is thought that 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 be 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 that is 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 that babies who are born with an umbilical hernia are three times more likely to develop Ewing’s sarcoma than others.

An umbilical hernia is where tissue pokes through the part of the abdomen near to the 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 sarcoma. It is unclear why this is the case.

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

Diagnosing bone cancer

If you visit Dr. B C Shah 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.

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

They can also refer you for a X-ray which can be a useful method of detecting bone cancer .

If no obvious cause can be found for your bone pain, it is likely that you will 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 that is 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 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. This does mean that many patients have to travel a long way 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 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 is performed under an 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. Dr. B C Shah  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.

Further testing

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

If you have an MRI scan, you will be asked to 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.

An MRI scan isn’t 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.

Staging

Once the tests described above 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 testing has revealed that 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 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 not 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 positive diagnosis

Being told that you have bone cancer can be a distressing, bewildering and frightening experience. Receiving that type of news can be upsetting at any age but it can seem particularly cruel if you are still in your teenage years, or if you are a parent of a child who has just been told that 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, visit Dr. B C Shah as there are a range of effective treatments, such as medication and counselling, that can help relieve feelings of depression.

Treatment for bone cancer

If you are diagnosed with bone cancer, it’s 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).

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)
• 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

Different types of bone cancer are usually treated in a similar way.

First, chemotherapy is given to try to shrink the tumour and to treat any cancer cells that may have spread.
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Following chemotherapy, surgery may be carried out 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.

In some cases – for example, if the cancer has spread beyond the bone into the surrounding blood vessels – reconstruction may not be possible and it may be necessary to amputate some, or all, of the limb. However, due to advances in diagnosis and treatment, only a minority of patients (around one-in-seven cases) need an amputation.

If cancer has spread to the lungs, surgery can also be used to remove the affected portion of the lungs.

Following surgery, further chemotherapy treatment may be given to destroy any remaining cancerous cells.

Radiotherapy may also be used for treatment and is particularly helpful for some types of cancer (such as Ewing’s sarcoma, for example).

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 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.

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:

• Feeling sick
• Being sick
• 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. Dr. B C Shah will be able to provide more detailed information about the specific risk to your fertility.

If you want 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 Dr. B C Shah 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 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.

Surgery

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:

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.

Amputation

Amputation may be required if:

• The cancer has spread beyond the bone into major blood vessels or nerves.
• The cancer has spread beyond the bone into your skin.
• You develop a serious infection after limb-sparing surgery.
• 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. Dr. B C Shah will understand the shock and fear that you, or your child, may be feeling and will be able to provide you with counselling and other support.

In some cases, Dr. B C Shah may be able to introduce you or your child to someone who has already 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 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.

Once your stump has healed and the swelling has settled after surgery, you can be fitted with an artificial limb. These are usually custom-made 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 to improve the skills you’ll 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.

Mifamurtide

A new medication called mifamurtide has recently been approved for 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 which are known to kill cancerous cells.

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

Mifamurtide is given using a process known as infusion, which means that it is slowly pumped into one of your veins over the course of an hour. This is usually done at your local hospital (you will be able to go home once the infusion has been completed).

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, but they are 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

Read the leaflet that comes with your medication for a full list of side effects.

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 have shown that your body is now free of cancerous cells, your various treatments will come to an end. But you’ll still need to attend regular follow-up appointments to check that the cancer hasn’t returned.

Followup appointments usually involve using X-rays and computerised tomography (CT) scan.

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

Your MDT will be able to provide more detailed information about the schedule of your follow-up appointments.

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 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.

Osteosarcoma

Six out of 10 people with localised osteosarcoma will live for 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 for at least five years after being diagnosed.

Ewing’s sarcoma

Seven out of 10 people with localised Ewing’s sarcoma will live for 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 for 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 for at least five years after a diagnosis.

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

Spindle cell sarcoma

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

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