What you need to know about bone sarcoma

Primary bone cancer, also known as bone sarcoma, is a rare type of cancer that develops in bone tissue. This is different to secondary bone cancer, which refers to cancer that develops in a different tissue or organ and spreads to the bones eg breast cancer that spreads to the ribs, spine, pelvis, or leg or arm bones. 

In the UK, only around 500 new cases of bone sarcoma are diagnosed each year. 

Bone sarcoma is part of a larger group of cancers called sarcomas: while bone sarcomas originate exclusively in bone tissue, soft tissue sarcomas, which make up the rest of this group of cancers, originate in any type of soft tissue that connects to or supports your organs ie blood vessels, fat, lymph vessels, nerves and muscles. A soft tissue sarcoma often presents as a painful lump that grows in size.

Here, we will focus on bone sarcoma, its symptoms and treatments, as well as who is most at risk. 

Who is at risk of bone sarcoma?

Bone sarcoma is very rare but when it does occur, it usually affects children and young adults. However, cases of bone sarcoma can also occur in the older age groups.

Certain rare inherited conditions can increase your risk of bone sarcoma. This includes Li-Fraumeni syndrome, hereditary multiple exostoses, neurofibromatosis type 1 and RecQ syndromes. The risk of bone cancer also increases if you are a survivor of heritable retinoblastoma. 

Types of bone sarcoma

There are five main types of bone sarcoma: osteosarcoma, chondrosarcoma, chordoma, Ewing sarcoma and giant cell tumours (GCTs). 

Osteosarcoma

This is the most common type of bone sarcoma in children and young adults aged 10–19 years old. Although it can develop in any bone, osteosarcoma most often affects the thigh bone (femur), shin bone (tibia) and upper arm bone (humerus). It accounts for around 30% of bone sarcoma cases. 

Chondrosarcoma 

This is the most common type of bone sarcoma in adults aged 30–60 years old and develops in cells in bones that form cartilage called chondrocytes. Chondrosarcoma can develop inside a bone or on the surface of a bone and most often affects the femur, pelvis, ribs and shoulder bones.

Chordoma 

This usually occurs in adults in their forties and fifties and accounts for around 6% of bone sarcoma cases. Around half of chordomas develop in the end part of the spine (sacrum); just over a third develop at the base of the skull; and the remaining cases develop in other parts of the spine.

Ewing sarcoma 

This is the most common type of bone sarcoma in children aged 0–14 years old and accounts for around 14% of bone sarcoma cases. It most often affects the femur, pelvis, shoulder bones and ribs. Ewing sarcoma can also affect soft tissues (extraosseous Ewing sarcoma), although this is rare. 

Stages of bone sarcoma

Bone sarcoma can be categorised into stages depending on the size of your tumour and how far the cancer has spread, which includes whether or not it has spread into your lymph nodes and/or to other parts of your body. 

Bone sarcoma can also be graded. This involves looking at the cancer cells under a microscope to determine how different (abnormal) they are compared to healthy cells. There are usually three grades:

• Grade 1 refers to cancer cells that look similar to healthy cells
• Grade 2 refers to cancer cells that look quite different to healthy cells
• Grade 3 refers to cancer cells that look very different to healthy cells

Low-grade bone sarcomas are slower-growing and less likely to spread than high-grade bone sarcomas.

Symptoms of bone sarcoma

The most common symptoms of bone sarcoma are unexplained pain in one of your limbs or near a joint, unexplained swelling, difficulty moving and pain when trying to bear weight. Pain is often worse when resting at night.

You may also often feel tired, have a fever, unintentionally lose weight and develop a bone fracture. 

Diagnosing bone sarcoma

If you notice any of the symptoms of bone cancer, see your GP. They will ask you about your symptoms and what makes them worse or better, as well as your medical history. They may also perform a physical examination and recommend that you have an X-ray and/or blood tests. 

Depending on your results, they may then refer you to a doctor who specialises in treating cancer (an oncologist). 

You may need further tests, such as an MRI scan or CT scan, and a biopsy where a tissue sample is collected. If your specialist suspects that you have bone sarcoma, you may need a bone biopsy, where a tissue sample is collected from the spongy tissue inside your bone. 

Treating bone sarcoma

Once you receive a diagnosis of bone sarcoma, you will be cared for by a multidisciplinary team, including an oncologist, orthopaedic surgeon, specialist cancer nurse and physiotherapist. 

The main treatment for bone cancer is surgery to remove the cancer, accompanied by chemotherapy and radiotherapy. The specific type of surgery, chemotherapy and/or radiotherapy you receive will depend on the type, grade and stage of your bone sarcoma. 

Chemotherapy is often performed both before and after surgery. Before surgery, chemotherapy can help shrink your tumour, which makes it easier for your surgeon to remove it. 

Radiotherapy is most often used to treat Ewing sarcoma or in cases where surgery isn’t possible or not all of the tumour could be removed surgically. 

As with any cancer, the earlier bone sarcoma is detected and treated, the better the outcomes. Around 55% of individuals with bone sarcoma live for 10 years or more. 

Life after treatment for bone sarcoma

In most cases, surgery is needed to treat bone sarcoma and consequently, you will need regular physiotherapy to help recover your mobility. 

If you have a prosthesis fitted as part of your surgery and you are still growing, you may need further surgery to replace the prosthesis or a procedure to extend a prosthesis specially designed to lengthen.  

Depending on the extent of your surgery, you may have a permanent disability, but you will be provided with support through physiotherapy and occupational therapy so you can engage in the activities that are important to you. 

You will also need regular follow-ups for 10 years after completing your treatment to monitor your health and check that the cancer hasn’t returned.