How is Ewing's sarcoma Diagnosed?
This information has been written for patients, their families and friends and the general public to help you understand more about Ewing's sarcoma: what it is and the different types. This information is produced in accordance with BCRT's information policy.
Some people are referred quickly for further tests or a second opinion, but often patients have to return to their GP at least three or four times. Primary Bone Cancers are very rare and many doctors will never come across a case.
One of the main symptoms is pain, and GPs sometimes suspect this of being ‘growing pains’ or a sports injury at first. This is probably because most people with Ewing's sarcoma are between 10 and 24 years old, and this is an age when a lot of people take part in sports.
Another condition that is sometimes misdiagnosed from the symptoms of Ewing's sarcoma is Osgood Schlatters disease. This condition is found in physically active adolescent boys and girls, and is caused by stress on the tendons that connect to the knee-cap. This also causes pain in the leg bones, similar to Ewing's sarcoma.
The Bone Cancer Research Trust (BCRT) is trying to find ways to make the time between the start of symptoms and getting the diagnosis much shorter. This is not a simple task and currently there is only a small amount of research on the subject. However, BCRT are participating in a national project to look at ways of reducing the length of time it takes to reach a diagnosis. Also, BCRT are working with the Royal College of GPs to fund a new online learning tool to help GPs to diagnose primary bone cancers.
Some patients go to their local hospital emergency department (A&E) or other health care centres. There is no scientific evidence available to examine whether patients presenting to their A&E are diagnosed any faster than patients who go to their GP. This is also an area where research is needed.
If a GP or doctor is concerned about the patient’s symptoms, there are national guidelines that they should follow:
Box 1. National Institute of Clinical Excellence (NICE) Guidelines
According to the National Institute for Clinical Excellence (NICE) guidelines for suspected bone cancer and sarcoma:
- If a primary healthcare professional has concerns about the interpretation of a patient’s symptoms and/or signs, a discussion with the local specialist should be considered.
- A patient who presents with symptoms suggesting bone cancer or sarcoma should be referred to a team specialising in the management of bone cancer and sarcoma, or to a recognised bone cancer centre, depending on local arrangements.
- Patients with increasing, unexplained or persistent bone pain or tenderness, particularly pain at rest (and especially if not in the joint), or an unexplained limp should be investigated by the primary healthcare professional urgently.
- A patient with a suspected spontaneous fracture should be referred for an immediate X-ray.
If a GP suspects primary bone cancer, they will normally ask for an x-ray of the bone and refer a patient for a specialist opinion, usually at a local hospital. They may also ask for some blood tests to look at the patient’s general health.
Again, according to the National Institute for Clinical Excellence (NICE) guidelines for a suspected bone cancer and sarcoma:
- If an X-ray indicates that bone cancer is a possibility, an urgent referral should be made.
- If the X-ray in normal but symptoms persist, the patient should be followed up and/or a repeat X-ray or bone function tests or a referral requested.
The guidelines state that if a primary healthcare professional is worried about any symptoms that could indicate primary bone cancer, they should ask a local specialist for advice. It may be more useful for the primary healthcare professional to contact a specialist at a bone cancer centre, where the staff are more used to diagnosing primary bone cancer.
If the x-ray shows that that the patient may have a primary bone cancer, then more tests are necessary. Some of these may also be done in a local hospital but at an early stage, patients should be referred to a Bone Cancer Centre for the completion of these tests.
In England, there are currently five Bone Cancer Centres, which specialise in the diagnosis and treatment of primary bone cancers. These centres are at Birmingham, Newcastle, Oswestry, Oxford and Stanmore.
In the Republic of Ireland, there are no specific Bone Cancer Centres. Patients are initially seen in their local hospital and subsequently referred to specialist hospitals in Dublin or Cork for further tests and, if necessary, for treatment.
Welsh patients usually travel to Oswestry or Birmingham for these specialist tests.
In Scotland there are five sarcoma centres and so patients travel to one of these centres for diagnosis if primary bone cancer is suspected. These centres are in Edinburgh, Glasgow, Inverness, Aberdeen and Dundee.
In Northern Ireland, patients are usually seen in Belfast.
Specialists in many different areas of medicine at hospitals in Ireland and at Bone Cancer Centres and the Regional Cancer Centres in the UK work together as a ‘Multi-Disciplinary Team’ (MDT). The Multidisciplinary Team works together to diagnose the patient.
- Specialist bone sarcoma surgeons.
- Specialist sarcoma oncologists (oncologists are doctors that look after people with cancer).
- Specialist sarcoma pathologist (pathologist are doctors that use laboratory techniques to diagnose disease).
- Radiologists (doctors that diagnose disease and conditions from looking at x-rays, or scans).
- Cytogeneticists (SY-tow-geh-NET-eh-sist), scientists that specialise in detecting damage to genes that help control the cell.
- Sarcoma cancer nursing specialists (sometimes called ‘CNS’) perform an essential role in treating and caring for primary bone cancer patients. These nurses are specially trained to look after sarcoma patients.
What tests are done?
When a person is referred to a Bone Cancer Centre, further tests will be done to find out more and to confirm whether the patient has primary bone cancer, and if so what kind of tumour it is. Patients are likely to have several different tests, including:
- X-rays are taken of the bone, including the joints above and below, and the radiographs (x-ray pictures) are studied. These x-rays may show swelling around the bone or areas of abnormal bone growth.
- A chest x-ray is sometimes taken to show whether the cancer has spread to the lungs.
Republic of Ireland patient information about radiology from the Irish Health Service Executive (X-rays, MRI Scans and CT Scans can be found here: Radiology (X-ray, MRI Scans, CT Scans) and here: HSE (x-rays)
- These tests help to check the patient’s general health as well as to look for a cause for the patients symptoms.
- A blood test involves taking a small sample of blood from a vein using a needle. The sample of blood is tested in a laboratory to check a patient’s general health and for levels of certain substances or chemicals in the blood. These include:
- Blood chemistry:
Us & Es stands for Urea and Electrolytes. This is checked to examine the levels of normal salts and urea and creatinine, which are waste products. This test can give clues to how well the kidneys are working.
Liver function tests (LFTs) to see how the liver is working. ALP stands for alkaline phosphatase (AL-kuh-line FOSS-fa-taze).
- Full blood count (FBC), which gives information about blood cells. The results show the counts of different types of blood cells:
Red blood cells – carry oxygen in the blood
White blood cells – are the cells that make up the immune system
Platelets – help blood to clot after an injury
Haemoglobin (HEE-moh-GLOW-bin) levels – show the oxygen-carrying capacity of the blood.
- Erythrocyte Sedimentation Rate (ESR) is a test to look for signs of inflammation. C-Reactive Protein (CRP) levels are also tested as CRP levels increase in inflammation.
- Blood chemistry: Us & Es stands for Urea and Electrolytes. This is checked to examine the levels of normal salts and urea and creatinine, which are waste products. This test can give clues to how well the kidneys are working.
- Scan of entire bone to gain more information about the tumour in the bone.
- MRI stands for magnetic resonance imaging. This type of scan is similar to a CT scan but magnetism and radio waves are used instead of x-rays to build up a very detailed 3-dimensional image.
- An MRI scanner is doughnut shaped; there is a short tunnel, which a motorised bed moves through during the scan.
- The MRI scanner can be quite noisy, and some machines have a CD player so that patients can listen to their choice of music during the scan.
- Sometimes an injection of a special dye, known as a contrast agent may be needed. This makes certain tissues show up more clearly and with greater detail on the scan.
- The results of the scan will be examined by a radiologist and a report will be produced.
- The patient has to lie very still during the scan and so sometimes very young patients can be anaesthetised during the scan.
There is more information on what to expect when undergoing MRI scans in the Cancer Research UK information on MRI scans
- CT stands for computerised tomography. They may also be called a CAT scan, which stands for Computerised Axial Tomography.
- The scanner takes x-rays from many different angles and a computer builds up a 3-dimensional picture of the body in great detail. The pictures show cross-sections of the inside of the body.
- CT scanning of the lungs shows up any secondary tumours where the cancer may have spread (metastasised).
- A CT scanner looks like a large doughnut with a bed for the patient to lie on. The bed will move slowly through the hole of the doughnut, while the machine takes the pictures.
- Before the scan, patients may be given a contrast medium. This contrast medium shows up in the scan so it helps to improve the image of some particular tissues and it can also help the radiologist tell the difference between blood vessels and other structures. The contrast medium is usually injected into a vein.
- The results of the scan will be examined by a radiologist and a report will be produced.
- Bone scans are used to look for abnormalities in bones. Patients who have a suspected primary bone cancer will sometimes have a full body bone scan.
- A tiny amount of harmless radioactive substance (radionuclide) is injected into the patient’s blood, which is then taken up by the bones fairly quickly (~2-4 hours).
- During the scan the radioactivity is detected by a specialised camera called a gamma camera. The radioactivity will collect more at areas of high activity (breakdown and repair) in the bone. These areas can be caused by a tumour.
- The areas of high activity picked up by the gamma camera are known as ‘hot spots.’
- The scans are usually carried out in hospital nuclear medicine departments.
- Patients will need to drink lots of fluids before the scan to help the radioactive substance travel to the bones quickly. The scanner looks like a bit like a CT scanner (doughnut shape). Patients lie on the bed, which travels through the doughnut.
- The results of the scan will be examined by a radiologist who will write the results in a report, which may take a few days to produce. Following the scan, the radionuclide will be passed completely from the body in the urine within 24 hours.
There is more information about bone scans in the Cancer Research UK information about bone scans.
- PET stands for ‘Positron Emission Tomography.’ Not all hospitals have PET scanners but they are increasingly being used in the diagnosis of cancer, and to show how well treatment is working.
- PET scans can examine the whole body, rather than a specific area. They can also detect how well treatments are working.
- Before the scan, a small injection of radioactive glucose (a radiotracer) called flourine18 will be given. Glucose is the fuel that cells use for energy. Cells that are very active such as cancer cells will take up more of this radioactive glucose than less active cells.
- The tracer will take around an hour to spread around the body. During the scan, which can last about an hour, the patient lies on a bed and the scanner passes over them. The scanner detects where the radiation is concentrated and produces images.
- When the body breaks down the radioactive glucose, particles called ‘positrons’ are released or emitted, the PET scanner detects the energy from the positrons and shows up as a 3-D image on a computer screen.
- Areas of high positron concentrations show up as a different colour and brightness on the image compared to areas of low positron concentration.
- The results of the scan will be examined by a radiologist
- A bone biopsy is a very specialised procedure that should only be performed by a specialist in orthopaedic surgery or sarcoma radiology. These biopsies should only be performed at a Bone Cancer Centre (see map).
- A biopsy is usually performed by the same surgeon who will operate on the patient’s cancer. Alternatively it can be performed by a specially trained radiologist.
- A biopsy involves taking a small sample of a lump or tumour so that a pathologist can examine the cells in the sample and determine whether the lump is cancerous or not.
- The biopsy sample can be taken using a needle, which is called a ‘needle biopsy’, or with a small operation, often referred to as an ‘open biopsy’. In both cases, a small amount of the lump is taken. Sometimes a scan is performed at the same time to make sure that the biopsy is taken from the right place.
- The biopsy is studied by a pathologist who can do different tests to work out what type of cells are in the sample, this is a process known as ‘histology’. This takes time and so the results of a biopsy can take a week or more.
There is more Information on what to expect when undergoing a in the Cancer Research UK information on bone biopsies
There is more information on what to expect when undergoing a bone marrow biopsy and aspirate in the Cancer Research UK information on bone marrow tests.
The biopsy enables doctors to be certain whether the patient has Ewing's sarcoma. This is important because other conditions can look like Ewing's sarcoma on x-rays and scans. This is known as a differential diagnosis. Other possible conditions can include:
- Eosinophilic granuloma (E-oh-SIN-oh-fill-ick GRAN-yule-oh-muh) – a benign (non-cancerous) bone tumour that is found mainly in children under the age of 10 years.
- Lymphoma (Limff-oh-ma) – a blood cancer that can cause bone destruction.
- Acute osteomyelitis (OS-tee-oh-MY-uh-LY-tis) bone damage caused by an infection inside the bone.
- Chronic osteomyelitis (OS-tee-oh-MY-uh-LY-tis) a more serious form of this bone infection.
- Osteosarcoma (ost-ee-oh-sar-coe-ma) – a different kind of primary bone cancer.
- Chondrosarcoma (KON-droh-sar-KOH-muh) – cancer of the cartilage that protects the outside of the bones.
At this point, most patients will be referred to their Regional Cancer Centre where treatment will start. This should be a specialist sarcoma centre or children's and young people's cancer centre.
Ewing's sarcoma information Version 2, June 2013
Last reviewed: June 2013
Next review due: June 2015
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. BCRT can 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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