Ewing sarcoma is a rare type of bone cancer.
Bone cancer is itself rare, with around 600 cases being diagnosed in the UK each year.
The three main types of primary bone cancer are chondrosarcoma, osteosarcoma and Ewing sarcoma.
Ewing sarcoma most commonly affects people who are 10 to 20 years old.
Ewing sarcoma usually occurs in bone. It most commonly develops in the:
However, the condition can also develop in the soft tissues surrounding the bone or joint. This type of Ewing sarcoma is known as extraosseous, which means outside the bone.
The symptoms of Ewing sarcoma will depend on the size of the cancer and where it is in the body.
The main symptoms are pain in the affected area that gets progressively worse, and swelling and tenderness.
Other possible, but less common, symptoms may include a high temperature (fever), severe tiredness and weight loss.
In some cases of Ewing sarcoma, there may not be any symptoms at all. However, as the tumour grows it could weaken the affected bone, increasing the risk of fracture.
Like other types of bone cancer, the exact cause of Ewing sarcoma is unknown.
As the condition tends to predominantly affect older children and teenagers, one theory is that it may be related to fast-growing bones.
The rapid growth spurt that happens during puberty may in some way make bone tissue more vulnerable to cancer.
Research has also found babies born with an umbilical hernia are three times more likely to develop Ewing sarcoma.
However, the increased risk is still small as only 1 in 110,000 children with an umbilical hernia will go on to develop the condition.
When you visit your GP because of bone pain, they'll ask you about your symptoms and examine the affected area.
Your GP may refer you for an X-ray to check for anything abnormal. If abnormalities are found, you'll be referred to an orthopaedic surgeon (a bone specialist).
They may recommend that you have a magnetic resonance imaging (MRI) scan to examine the area in closer detail.
A bone biopsy may be carried out to confirm whether cancer is present. A fine needle is used to remove a sample of bone marrow from the affected area so it can be examined under a microscope. It can either be carried out using a general anaesthetic or a local anaesthetic.
Like all types of cancer, the earlier Ewing sarcoma is diagnosed, the easier it is to treat.
Ewing sarcoma is usually treated with a combination of chemotherapy, surgery and radiotherapy.
Chemotherapy may be used before surgery to shrink the tumour and make it easier to remove.
Ewing sarcoma also responds well to radiotherapy. It can be used to shrink the tumour after chemotherapy, and before or after surgery to lower the risk of the cancer returning.
In some cases, surgically removing Ewing's sarcoma can be difficult – for example, if it develops in the pelvis. In this case, radiotherapy may be used as the main treatment.
If surgery is recommended, the type you'll have will depend on:
You may have surgery to remove part or all of the bone affected by cancer. This type of surgery is known as resection.
Limb-sparing surgery involves removing the area of bone on an arm or leg where the cancer is growing. A piece of metal (prosthesis) or a bone graft will be used to replace the piece of bone that is removed.
In some cases, your whole arm or leg may need to be removed (amputation). This may be the only option to prevent the cancer returning if it's spread into the tissues surrounding the bone.
For more information about the types of treatment for bone cancer, visit the Cancer Research UK website.
If you're diagnosed with bone cancer, it can be upsetting and frightening. Knowing as much about the type of cancer you have and the best treatment options will help you make decisions.
The majority of people who have surgery for bone cancer have a limb-sparing procedure.
However, for some people, having their limb amputated may be the best option. This can be a very difficult situation to cope with and you may experience emotions such as grief and bereavement.
It's important to seek help and talk about how you're feeling. You could talk to your partner, family, friends or the healthcare professionals in charge of your care. Talking to others will help you come to terms with your condition and treatment.
There are also a number of practical issues you'll need to deal with, such as financial matters and coping with day-to-day activities such as school, college or work.
If you or your child has Ewing sarcoma, your clinical team will pass information about you/your child on to the National Congenital Anomaly and Rare Diseases Registration Service (NCARDRS).
This helps scientists look for better ways to prevent and treat this condition. You can opt out of the register at any time.