Definition

Bone sarcoma is a cancer that starts in the bone. Sometimes a bone sarcoma is called a primary bone cancer. It is extremely rare and makes up only 0.2% of all cancer diagnoses in England. 

There are on average, 500 diagnoses in the UK each year. Bone sarcoma can affect any bone in the body but the most common area it affects is the legs. 

The four most common types of bone sarcoma are: 

Chondrosarcoma
Chondrosarcoma develops in the cartilage cells. It mostly affects adults above the age of 40 and the most common sites are the upper arm, pelvis and thigh bone. They make up 37% of all bone sarcoma diagnoses.

Osteosarcoma
Osteosarcoma is mostly diagnosed in teenagers and young people. However, it can also affect older adults. It mostly affects the knee, thigh bone, shin bone or upper arm. They make up about 30% of all bone sarcoma diagnoses.

Ewing’s sarcoma
Ewing's sarcoma makes up 14% of all bone sarcoma diagnoses. It most commonly affects teenagers and young adults with the pelvis, thigh bone and shin bone being the most commonly affected areas. In rare cases, Ewing’s sarcoma can develop in the soft tissue around the bone. This is called soft tissue sarcoma. It is sometimes called extraosseous sarcomaextra means outside, osseous means bone. Ewing’s sarcoma can also start in subcutaneous tissue, which means beneath the skin. 

Chordoma
Chordoma makes up only 6% of all bone sarcoma diagnoses and most commonly affects adults in their 40’s and 50’s. The majority of chordomas (50%) arise in the sacrum (the bottom of the spine). However, 30% arise at the base of the skull and the remaining 20% arise in other locations in the spine.

Signs and symptoms

Symptoms of bone sarcoma can vary depending on the size and location of your tumour.

  • Bone pain, particularly occuring at night
  • A mass or swelling
  • Restricted movement in a joint

Symptoms can sometimes be confused with more common problems such as a sports injury or in children and young people, or growing pains. 

Types of diagnostic scans

X-ray

Uses x-radiation to take images of dense tissuses inside the body such as bones or tumours.

CT

The Computer Tomography (CT) scan takes a number of x-rays to make a 3D image of an affected area.

PET

The Positron Emission Tomography (PET) scan shows up changes in tissues that use glucose as their main source of energy – for example, the brain or heart muscle. It involves an injection of a very small amount of a radioactive drug into the body. The drug travels to places where glucose is used for energy and shows up cancers because they use glucose in a different way from normal tissue. 

MRI

Magnetic Resonance Imaging (MRI) uses magnets to create an image of the tissues of the body.

Bone scan

Uses radioactive chemicals called radionuclides which are injected, swallowed or breathed into the body, to take images of bones. 

Histopathology

Examination of a tissue sample by a pathologist under a microscope to identify disease.

Blood test

Laboratory analysis of a blood sample.

Understanding the diagnosis

A diagnosis of bone sarcoma usually starts with a visit to your GP who will refer you for an x-ray. If a bone sarcoma is suspected following the results of the x-ray, your GP will refer you to a specialist bone sarcoma centre. 

Your symptoms will then be investigated further by a specialist doctor using a series of tests that may identify sarcoma. Tests may include: 

  • Physical examination 
  • A scan – taking pictures of the inside of the body using ultrasound, x-ray, CT, PET or MRI
  • A biopsy – taking and testing a tissue sample

A diagnosis of bone sarcoma should be confirmed by a specialist sarcoma pathologist who will identify the type of sarcoma and the stage and grade of the tumour. Identifying the stage and grade of a cancer means your doctor can advise on the best course of treatment for you. It also describes the cancer in a common language which is useful when your doctor is discussing your case with other doctors or health professionals. The stage of a cancer is measured by how much it has grown or spread, which can be seen on the results of your tests and scans. The results from a biopsy can tell the grade of the cancer. 

Grading

Low-grade means that the cancer cells are slow-growing and look quite similar to normal cells. They are less aggressive and less likely to spread. 

High-grade means the cancer cells are fast growing and look very abnormal. They are more aggressive and more likely to spread. 

Staging

Stage 1

The cancer is low-grade and has not grown outside the bone. Stage 1 is further divided into:

  • 1a The cancer is low-grade and is still completely within the bone it started in.
  • 1b The cancer is low-grade and has grown through the bone wall.

Stage 2

The cancer is high-grade and has spread beyond the bone. Stage 2 is further divided into:

  • 2a The cancer is high-grade and is still completely in the bone it started in.
  • 2b The cancer is high-grade and has grown outside the bone wall.

Stage 3

The cancer may be any grade and has spread to another part of the body, such as the lungs. 

Treatment

The National Institute for Health and Care Excellence (NICE) recommends that anyone with sarcoma should be referred to a specialist sarcoma team for diagnosis and treatment. There are five national bone sarcoma centres in England and Wales where all bone sarcoma patients should be treated. These are located in Newcastle, Manchester & Oswestry, Birmingham, Oxford and London.

People in Northern Ireland will be treated in Belfast.

People in Scotland will be treated in Glasgow, Edinburgh, Aberdeen, Dundee and Inverness. This may mean you will have to travel some distance for your treatment. Some treatments like radiotherapy or chemotherapy may be given in your local hospital under the supervision of the specialist sarcoma centre.

Your case will be managed by a team of experts from a wide range of health care professions called a multidisciplinary team (MDT). Your MDT will include your key worker or sarcoma clinical nurse specialist, surgeon and other healthcare professionals involved in your care. 

Types of treatment

The treatment you will receive depends on the type of bone sarcoma you have and which part of the body is affected. Your MDT will discuss your case and your doctor or nurse will talk you through your options so you are included in deciding what treatment is best for you. 

Click through for the treatment options for each of the following kinds of bone sarcoma.

After treatment

After treatment you will have regular follow up appointments for several years. These appointments are designed to look for signs of your cancer returning or signs that it has spread
to another part of the body. You should receive a follow-up schedule from your sarcoma clinical nurse specialist. The usual practice will include:

• A chance to discuss symptoms
• An assessment of the function of any reconstruction, such as a prosthesis or bone graft, and a look at any complications that may have arisen following surgery
• A physical examination and CT or MRI to look for any signs of your sarcoma returning
• A chest x-ray to look for any secondary cancers occurring in the lungs

Recurrence

Sarcoma cancer can reappear in the same area after treatment of a previous tumour. This is called a local recurrence. 

If the cancer does reappear, it is important to get treated as quickly as possible. This could involve further surgery, radiotherapy and/or chemotherapy. Your treatment will be assessed on an individual basis.

It is useful to check for recurrences yourself through self-examination – your doctor or sarcoma clinical nurse specialist can tell you what to look for. If you are worried about your cancer returning contact your doctor or sarcoma clinical nurse specialist. They may decide to bring forward the date of your follow-up appointment to investigate your concerns.

What if my cancer spreads?

A recurrence of cancer may appear in other parts of the body. This is called a metastasis or secondary cancer. In people with bone sarcoma, these secondary cancers may appear in the lungs. A chest x-ray is taken at follow-up appointments to look for secondary cancers in the lung. Treatment for secondary cancers may involve surgery, radiotherapy or chemotherapy as appropriate. Your treatment will be assessed on an individual basis.