Primary Bone Cancer Information

This information has been written for patients, their families and friends and the general public to help them understand more about primary bone cancer and how it is diagnosed and treated.

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What is Primary Bone Cancer?

Primary bone cancer, sometimes known as a bone sarcoma, is a cancer that starts in a cell found in the bone.

Sarcoma is the name given to a cancer that starts in the connective tissue. The connective tissue plays a supportive role in the body and includes tissues such as the bone, cartilage, muscle and fat.

Primary bone cancer can develop in any bone in the body but most commonly is found in the long bones of the lower body (34%), such as the thigh (femur) or shin bone (tibia).

Every year, around 562 people in the UK alone will develop primary bone cancer at an average rate of 2 cases per day. In 2017 the total number of cancer cases in UK was 363,484. Primary bone cancer represents 0.155% (rounded up to 0.2%) of all cancer diagnosis in UK.

When combining known primary bone cancer incidences, there are approximately 52,000 cases of primary bone cancer cases reported each year worldwide, and a diagnosis of primary bone cancer is made every 10 minutes somewhere in the world.
Cancer cells can separate from a tumour elsewhere in the body and spread to the bones. This is called secondary bone cancer and its management can be very different to primary bone cancer.

What are the Symptoms of Primary Bone Cancer?

Symptoms of primary bone cancer can be very general and often appear similar to the symptoms of sports injuries, growing pains or many other common conditions such as tendonitis or arthritis. They may also vary depending on the location and the size of the tumour.

Yes, there are several types of primary bone cancer. 85% of all primary bone cancers diagnosed are of the 4 most common types, which are:

Osteosarcoma, Ewing Sarcoma, Chondrosarcoma and Chordoma.

There are also several less common forms of tumours that arise in the bone, these are:

Spindle Cell Sarcoma of the Bone: such as Pleomorphic Undifferentiated, Sarcoma (previously known as Malignant Fibrous Histiocytoma), Adamantinoma
Angiosarcoma of the Bone and Giant Cell Tumour of the Bone (a non-cancerous tumour).

Bone marrow is found within the bone but cancers which develop in the bone marrow (myeloma and leukaemia) are not primary bone cancers. Lymphomas start in the lymph nodes and then may infiltrate the bone marrow. The bone marrow is part of the lymphatic system, so the disease can originate there. For more information on cancer types of the bone marrow please visit Cancer Research UK, Myeloma UK or Blood Cancer UK.

Who does Primary Bone Cancer Affect?

Primary bone cancer is unusual in that it affects a wide range of age groups. It can affect children, adolescents, young adults and can also occur in elderly people; whereas the majority of other primary cancers occur mainly in older adults (1,2).
The four common forms of primary bone cancer in terms of their incidence and anatomical sites are:

Chondrosarcoma
Most common in adulthood
Highest incidence: 30-60- years old
Common sites: long bones, pelvis and ribs

Osteosarcoma
Most common in children and young adults
Biphasic incidence peak: 15-19-years old, 70-89-years old
Common sites: long bones, especially around the knee

Ewing sarcoma
Second most common in children and young adults
Highest incidence: 10-20-years old
Common sites: long bones, pelvis, ribs, vertebrae
Males are at a slightly higher risk of developing most types of primary bone cancer than females

How is Primary Bone Cancer Diagnosed?

Primary bone cancer is rare and often misdiagnosed as other conditions e.g sports injuries, growing pains or arthritis. Increased awareness of primary bone cancer amongst GPs, healthcare professionals and patients, is key to earlier diagnosis and improved outcomes.

If you have symptoms of primary bone cancer, you will usually start by seeing your GP, visiting the local hospital emergency department (A&E) or another health professional such as a physiotherapist or a hospital doctor.

As primary bone cancers are rare and many GPs may never have seen a case before, it is quite common for patients to visit their GP 3 or 4 times before receiving a referral. This may also mean a referral is sometimes made by Accident & Emergency Departments or another health professional.

Going to the GP
Your GP will examine the area, look for any lumps or swelling and ask you about your symptoms and the pain you are experiencing:
Does it come and go?
Is it worse at night?
Is it relieved with painkillers?
Is there a lump or swelling that can be seen or felt?
Is it tender to touch?
Do you have an unexplained limp, stiff joints, or reduced mobility?
Does the affected area bruise easily?
Are you experiencing tiredness, fever, weight loss or loss of muscle tone?

Your GP may refer you for blood tests and an X-ray of the affected areas. If the X-ray shows an abnormal area or primary bone cancer is suspected, you will be referred to a specialist bone sarcoma multidisciplinary team (MDT) for further assessment.

To help GPs, the National Institute for Health and Care Excellence (NICE) has produced a Suspected Cancer: recognition and referral guideline (NG12) (3). The guideline says the following:

Children, teenagers and young adults with unexplained bone swelling or pain should have an urgent X-ray within 48 hours. If the X-ray suggests a possible bone cancer, your GP should refer you to a specialist within 48 hours.
Adults should be seen by a specialist within 2 weeks if the results of an X-ray suggest a bone cancer.

If the X-ray is normal but symptoms persist, the patient should be followed up and/or a repeat X-ray or MRI scan should be carried out within 2 weeks (adults) or within 48 hours (child).

What happens if primary bone cancer is suspected?

If a primary bone cancer or bone tumour is suspected, you will be referred to a bone cancer surgical centre for diagnosis although some head and neck, spinal and paediatric biopsies may take place in a bone cancer MDT-approved specialist centre. You will usually be referred to the bone cancer surgical centre closest to where you live.

The bone cancer centres in the UK and Ireland are:

There are five centres in England:
Birmingham
Newcastle
Oswestry
Oxford
Stanmore

Wales
Patients in Wales travel to Oswestry or Birmingham bone sarcoma surgical centres.

Scotland
There are five centres in Scotland:
Edinburgh
Glasgow
Aberdeen
Dundee
Inverness

Republic of Ireland
There are no specific bone sarcoma surgical centres in the Republic of Ireland at the moment. Patients are referred to specialist hospitals in Dublin or Cork for further tests or treatment.

Northern Ireland
Patients are seen in Belfast.
At the bone sarcoma surgical centre, you will undergo a series of diagnostic tests and investigations to check your organ function before any treatment is started. Each patient, and tumour subtype, may undergo different diagnostic tests that are tailored to the needs of the individual patients. Your test results will be discussed by the specialist bone sarcoma MDT based at the bone sarcoma surgical centre who work together to reach a diagnosis.

The diagnostic tests are:
X-ray
An X-ray is a procedure used to produce images of the inside of the body using radiation. This is likely to be the first test used to diagnose bone cancer. X-rays are studied to detect swelling, abnormal bone growth and the breakdown of an area of bone. A chest X-ray is sometimes taken to see if the cancer has spread to the patient’s lungs.
For more information on X-rays, please click here.

CT Scan
A CT (Computerised Tomography) scanner is a machine that takes a number of X-ray pictures from different angles to form a 3D image of the area of the body being looked at. The CT scan helps doctors to understand in more detail the size of the tumour, the exact location of the tumour and whether there are signs of the tumour having spread elsewhere in the body. A CT scan should take around 10-20 minutes. Additional CT scans of the chest can show if the cancer has spread to the patient’s lungs. For more information on CT scans, please click here.

MRI Scan

An MRI (Magnetic Resonance Imaging) scan is similar to a CT, but magnetism and radio-waves are used instead of X-rays. These scans build up a very detailed 3D image to give more information about the tumour. The MRI provides a picture of where the tumour is in the bone, and whether it has grown out to involve the surrounding soft tissues. MRI scans help doctors decide on the right treatment plan for an individual.
For more information on MRI scans, please click here.

PET Scan
PET (Positron Emission Tomography) can be used to diagnose cancer. These scans examine the whole body rather than one specific area. This allows doctors to determine the location and size of the tumour, if the tumour has spread elsewhere in the body and how well the patient is responding to treatment. As this scan examines the whole body it can take around an hour to complete and requires the patient to lie flat on a bed as the scanner passes over them.
Before the scan, the patient will receive an injection of harmless radioactive glucose (known as a radiotracer). This radiotracer is taken into cells that are active. As cancer cells grow and divide more rapidly than healthy cells, they take up the radiotracer more readily than healthy cells. When the radiotracer has been taken up by the cancer cells, these cells break down this substance and release particles known as positrons. It is the released positrons that can be located during the PET scan to create a 3D image showing varying brightness levels on the scan relating to a higher number of cancer cells in that area.
PET scans are often combined with CT scans (PET-CT) to create a more detailed picture.
For more information on PET scans, click here.

Isotope Bone Scan
Bone scans are used to look for abnormalities in the bones. Patients who are suspected of having primary bone cancer will often have a full body bone scan. The scan is carried out using a gamma camera, which detects areas of radioactivity. Much like CT and MRI scans, patients lie flat on a bed while the scan is taking place.

Before having the scan, the patient will have an injection containing a tiny amount of a harmless radioactive substance (known as a radionuclide) into the blood. This substance is taken up by the bones over a few hours and can be detected by the gamma camera. The radionuclide will collect in larger amounts in areas of high activity (where breakdown and repair of the bone is occurring) and these areas are referred to as ‘hot spots’, which appear as darker areas on the scan image. These represent areas which may be cancerous or represent another medical condition, such as arthritis.
For more information on bone scans, please click here.

Blood tests
Blood tests can be carried out to assess the general health of a patient. This sample is tested in a laboratory to show the levels of certain substances in the blood, to check for infection and to help diagnose many different conditions and diseases – including cancer.

The blood tests carried out to help diagnose primary bone cancer may include:
A Full Blood Count (FBC): a full blood count shows the number of each cell type (red cells, white cells, platelets, haemoglobin) in the patient’s blood to check that the levels are normal and healthy

Urea and Electrolytes (U&Es): these tests determine how well the kidneys are working. Urea is a waste chemical removed by the kidneys and electrolytes include sodium, potassium and chloride. The levels of these substances may alter during an illness or disease treatment and it is important they remain balanced for the body to function normally.

C-Reactive Protein (CRP): this protein is produced by the liver when there is an increase in inflammation in the body, a process which occurs during cancer
Erythrocyte Sedimentation Rate (ESR): this test looks for any signs of inflammation in the body and detects the presence of any abnormal cells in the blood.

Alkaline Phosphatase (ALP): alkaline phosphatase is mostly produced by the liver but can also be made in the bones. This enzyme is measured in patients with suspected osteosarcoma to see if there is any weakening or destruction of the bones.

LDH– Lactate dehydrogenase: LDH is an enzyme the body uses during the process of turning sugar into energy for your cells to use. LDH is found in many of the body’s tissues and organs including the muscles. Usually the concentration in the blood is low because it stays within the tissues’ cells. When the cells are damaged/ destroyed, they release LDH into the bloodstream, causing blood levels to rise. The LDH test is used to identify the location and severity of tissue damage in the body. It is also sometimes to monitor how far certain conditions have progressed (i.e. kidney disease, liver disease and some types of cancer). LDH is also a marker of hypoxia in cells but in not a marker of cancer exclusively as it can be indicative of other conditions e.g. kidney problems. It may also not pick up smaller tumours.

Ca2+ – Blood Calcium (total calcium): This test monitors a range of conditions relating to bones, heart, nerves and kidneys. Calcium is one of the most important minerals in the body (99% of calcium is found in bones with the rest circulating in the blood). For more information on blood tests, click here.

Bone biopsy
Tumours found in the bone may be benign (non-cancerous) or malignant (cancerous). To find out whether the tumour is benign or malignant, a biopsy sample must be taken and assessed.

A bone biopsy is a specialist procedure performed at the bone cancer treatment centre. A small sample of the tumour will be taken and examined under a microscope by a histopathologist to see if the patient’s tumour is cancerous, and if so, the subtype of primary bone cancer. The biopsy may be a ‘needle biopsy’ or an ‘open biopsy’:

Needle Biopsy: a needle is inserted into the tumour to draw out a small amount of tumour tissue (this may be done under local anaesthetic). Often, in order to know exactly where to take the sample from, this test is carried out alongside an X-ray or CT scan to guide the doctor.

Open Biopsy (or surgical biopsy): is used less frequently than a needle biopsy. This form of biopsy is carried out during a small, minor, operation to remove a small piece of tumour while under general anaesthetic. This test tends to be used if a needle biopsy does not provide a diagnosis and the doctor needs to investigate further.

Bone marrow biopsy: On occasions patients with Ewing sarcoma will have a bone marrow biopsy and aspirate taken to see whether the Ewing sarcoma may have spread to the bone marrow. This is not always carried out if the patient has had a

PET scan.
Results from a biopsy can take up to two weeks but this may vary.
Questions to ask your medical team at the time of diagnosis
It may be a good idea to write down the questions you would like to ask your doctor, or symptoms you wish to tell them about, before your appointment. You may also benefit from taking a notebook to write down key things to remember, or even better, take a companion who can do this for you.

We have put together a list of questions you may find useful to ask your medical team during your appointments:
Where can I find more information about my cancer type?
Do you know the stage of my cancer and has it spread elsewhere in my body?
How can I manage my symptoms and what shall I do if they become worse?
Are there any activities I should avoid?
Which tests will I be having and where will I need to go to have these tests?
How will I receive the results from my tests? How long could it take for my results to be communicated?
Will I need to make any changes to my day-to-day life, diet or exercise following my diagnosis?
Who can I contact if I have any further questions, and will I be able to do so during out-of-office hours?
Who can I talk to if I am feeling distressed or overwhelmed with my diagnosis?
If you have any further questions about your diagnosis or require any further support contact us.

How is Primary Bone Cancer Graded and Staged?

The results of the diagnostic tests help your doctor to work out:
The grade of the cancer- how the cancer looks under a microscope which can be used to predict how the cancer might grow and spread.

The stage of the cancer – how big it is and whether it has spread outside of the bone. Identifying the stage and grade of the cancer enables your doctor to advise on the best course of treatment for you.

The grading and staging of primary bone cancer can seem complicated. There are 2 main systems for staging your primary bone cancer and the grading is slightly different for each system.

Grading Primary Bone Cancer
Doctors grade cancer cells according to how the cells look under a microscope. The grade of the bone cancer gives your specialist a guide to how the cancer may behave. The most common grading system for bone cancer uses two grades:

Low grade cancers have cells that look slightly abnormal. These cancers usually grow slowly and are less likely to spread.

High grade cancers have cells that look very abnormal. These cancers are likely to grow more quickly and are more likely to spread.

Staging Primary Bone Cancer
The stage of a cancer tells your doctor how big it is and whether it has spread.
Your doctors and surgeons might use one of 2 different systems to stage your bone cancer. These are:

the Enneking stages (surgical)
the TNM stages

They should explain which one they are using and what this means for you. The most used systems are Enneking and TNM.

The Enneking staging system
As well as the size of the tumour and if it has spread, the Enneking staging system also describes the grade of the cancer. It helps your surgeon decide how much bone to remove during surgery.

Stage 1
Stage 1 bone cancer is low-grade. It has not spread beyond the bone. Stage 1 is divided into:
Stage 1A – the cancer is completely inside the bone it started in. The cancer may be pressing on the bone wall and causing a swelling but it has not grown through it.
Stage 1B – the caner has grown through the bone wall.
Stage 2
Stage 2 bone cancer is high-grade. It has not spread beyond the bone. Stage 2 is divided into:
Stage 2A – the cancer is completely inside the bone it started in.
Stage 2B – the cancer has grown through the bone wall.
Stage 3
Stage 3 bone cancer may be any grade. It has spread to other parts of the body, such as the lungs.

TNM staging system
TNM stands for Tumour, Node and Metastases:
T – describes the size of the tumour. T (tumour) is divided into 3 stages – T1 to T3:
T1 means the tumour is 8cm or less at its widest point.
T2 means the tumour is more than 8cm.
T3 means a high-grade tumour where there is more than one area of cancer in the same bone.
N – describes whether the cancer has spread to lymph nodes. N (nodes) is divided into 2 stages – N0 and N1:
N0 means there are no cancer cells in lymph nodes close to the tumour.
N1 means there are cancer cells in nearby lymph nodes.
M – describes whether the cancer has spread to another part of the body (metastatic or secondary cancer). M (metastases) is divided into 3 stages – M0, M1a and M1b:
M0 means the cancer has not spread to any other part of the body.
M1a means the cancer has spread to your lung.
M1b means the cancer has spread to other areas of the body apart from the lung.

In the TNM system, bone cancer cells are graded from GX to G4:
GX means the grade cannot be assessed
Grades 1 and 2 are low grade tumours
Grades 3 and 4 are high grade tumours

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Version 2 – Information kindly supplied with permission by The Bone Cancer Research Trust.
Information is currently under review

The authors and reviewers of this information are committed to producing reliable, accurate and up to date content reflecting the best available research evidence, and best clinical practice. We aim to provide unbiased information free from any commercial conflicts of interest. This article is for information only and should not be used for the diagnosis or treatment of medical conditions. We may be able to answer questions about primary bone cancers, including treatments and research but we are unable to offer specific advice about individual patients. If you are worried about any symptoms please consult your doctor.

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