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 how Ewing's sarcoma is diagnosed. The information looks at the tests that may be carried out and where they will be done.

Going to the doctor

People report a variety of experiences when they seek medical advice about their symptoms. Most people with worrying symptoms go to their General Practitioner (GP). 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 GPs will never come across a case. The symptoms of Ewing's sarcoma are general and one of the symptoms, pain, may be suspected of being 'growing pains' or a sports injury at the first visit. This is probably because most people with Ewing's sarcoma are between 10 and 20 years old, and this is an age when a lot of people take part in sports.

The Bone Cancer Research Trust 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 hoping to fund more research and have recently put out a call for research proposals aimed at finding out why delays in diagnosis can occur, and what could be done to improve the time to diagnosis.

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 specializing in the management of bone cancer and sarcoma, or to a recognized 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 a 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 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 is normal but symptoms persist, the patient should be followed up and/or a repeat X-ray or bone function tests or a referral requested.

If a GP suspects a primary bone cancer, they will normally ask for an x-ray of the bone or refer the patient for a specialist opinion. This is normally done at a local hospital or clinic. They may also ask for some blood tests to look at the patient's general health.

If the x-ray shows that that the patient might 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.

Going to a Bone Cancer Centre for more tests

Bone cancer centres are specialist centres. They have a group of healthcare specialists who are experts in the diagnosis and treatment of bone cancer. In England, there are currently five Bone Cancer Centres where the surgical treatment of bone cancer is carried out. These are at Birmingham, Newcastle, Oswestry, Oxford and Stanmore.

In Wales, Scotland and Northern Ireland the organisation is slightly different: patients from Wales often go to Oswestry or Birmingham for surgical treatment. In Scotland, patients are seen in one of three centres in Glasgow, Edinburgh and Aberdeen. In Northern Ireland, patients are seen in Belfast. Other treatments such as chemotherapy and radiotherapy can often be given closer to home, see Figure 4.

In the Republic of Ireland, there are no specific 'Bone Cancer Centres'; patients are initially seen by their local hospital and subsequently referred to specialist hospitals in Dublin and Cork for further tests and treatment (see, 'going to a Regional Cancer Centre for more tests and starting treatment,' for further details).

More tests are carried out at the Bone Cancer Centre and then the specialist team work out the beginning of a treatment plan. The tests patients are likely to have include

  • Radiographs (pictures) of affected bone including the joints above and below. These x-rays may show swelling around the bone or areas of abnormal bone growth.

  • An x-ray of the chest may show if the cancer has spread to the lungs.

UK patient information on x-rays and what to expect can be found here: UK Patient Information about X-rays

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)

Blood Tests
  • 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 or if a patient has one from a central line, PICC or Portacath®. The sample of blood is tested in laboratories to check a patient's general health and for levels of certain substances or chemicals in the blood.

Types of tests may include:

  • Checking the blood chemistry (Us & Es), to examine the amounts (levels) of dissolved salts as well as urea and creatinine (waste products). This test can give clues to how well the kidneys are working.

  • Full blood count (FBC), which gives information about blood cells; how many red blood cells, white blood cells, (and totals of each type), platelets and levels of a substance called haemoglobin (HEE-moh-GLOW-bin).

  • Tests to see how the liver is working (LFTs).

  • Tests to look for signs of inflammation (CRP, ESR), these tests can help to detect infection and disease processes that cause inflammation.

  • Test (ALP) to check the level of a substance called alkaline phosphatase (AL-kuh-line FOSS-fa-taze) in patients with suspected osteosarcoma.

Patient information about blood tests can be found here: Blood Tests.

MRI Scan
  • 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 'do-nut' shaped; there is a short tunnel, which a motorised bed moves through during the scan.

  • The MRI scanner can be quite noisy

  • 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.

Information on what to expect when undergoing a MRI scan can be found on NHS direct: UK Patient Information about MRI Scans

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).

Figure 2

  • CT scanning of the lungs to look for any spread of the cancer (metastases).

  • CT stands for computerised tomography. They may also be called CAT scans, 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 slices of the inside of the body.

  • A CT scanner looks like a large 'do-nut' with a bed for the patient to lie on. The bed will move slowly through the hole of the 'do-nut,' while the machine takes the pictures.

  • Before the scan, patients may be given a contrast medium. This contrast medium 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.

Information on what to expect when undergoing a CT scan can be found on NHS direct: UK Patient Information about CT Scans

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).

Figure 3

Bone Scan
  • Bone scans are used to look for abnormalities in bones. Patients that have a suspected primary bone cancer will probably have a full body bone scan.

  • A tiny amount of 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. This could 'suggest' a cancer or secondary cancers (metastases).

  • 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 ('do-nut' shape). Patients lie on the bed, which travels through the 'do-nut.'

  • The results of the scan will be examined by a radiologist and a report will be produced. This may take a few days. Following the scan, the radionuclide will be passed completely from the body in the urine within 24 hours.

Patient information on bone scans can be found here.

PET Scan
  • PET stands for 'Positron Emission Tomography.' There are not many hospitals that have PET scanners and they are not often used in the diagnosis of bone cancers.

  • 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

Information on what to expect when undergoing a PET scan can be found here.

Biopsy performed by a specialist oncological orthopaedic surgeon
  • A biopsy involves taking a sample of a lump or tumour for a pathologist to look at under a microscope. A pathologist is a doctor who uses laboratory techniques to diagnose disease. This enables them to work out what type of cells the tumour is made up of and whether or not it is cancerous. This is known as the 'histology.' The techniques involved in determining the histology include electron and light microscopy, molecular biology and a technique called immunocytochemical (IMM-yoo-no-SY-toh-chemical) staining.

  • Electron and light microscopes are used to look at:

    • Cell size and cell shape,
    • The size of the nucleus in the cell
    • The amount of cells dividing (rate of mitosis)
    • The number of cell structures (organelles) that are present


  • Immunocytochemical staining is used to detect specific proteins on and inside cells.

    • The small-blue-round cells of Ewing's sarcoma can appear similar to other types of cancer (the cells appear as blue from the stain used by a pathologist when examining cells).
    • Techniques using special stains and other laboratory tests are needed to show the differences between Ewing's family of tumours and other round cell tumours. This is important because different types of round cell tumours have different treatments.

  • Molecular Biology techniques such as the Reverse Transcriptase Polymerase Chain Reaction (rtPCR) help to find the chromosome translocations specific to Ewing's sarcoma and other gene mutations.

  • The biopsy sample can be taken using a needle, 'needle biopsy' or in surgery, 'surgical biopsy.' In both cases, a small amount of the lump is taken. Sometimes doing a scan at the same time may help the surgeon find the lump to take the sample.

  • The results of a biopsy can take a week or more.

Further information on biopsies and what to expect when undergoing a biopsy can be found on NHS direct: UK Patient Information about Biopsies

Republic of Ireland patient information about biopsies from the Irish Health Service Executive can be found here: HSE Patient Information about Biopsies

The biopsy enables doctors to be certain whether the patient has osteosarcoma. This is important because other conditions can look like osteosarcoma 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)
  • Lymphoma, (bone destruction from)
  • Acute osteomyelitis (OS-tee-oh-MY-uh-LY-tis)
  • Chronic osteomyelitis
  • Some forms of osteosarcoma
  • Chondrosarcoma (KON-droh-sar-KOH-muh)

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. See Figure 4.

Going to a Regional Cancer Centre for more tests and starting treatment

When patients arrive at their Regional Cancer Centre, more tests will be carried out to show how well organs such as the kidneys, heart, liver and ears are working. Tests may include blood tests and echocardiograms (heart). The measurements from these tests show doctors if these organs are working normally. This gives the doctors a 'baseline' to compare how well these organs are working as your treatment goes on.

The drugs used to treat Ewing's sarcoma can affect these organs, so the tests are repeated during treatment. The measurements taken from the repeated tests are compared to the baseline; this helps the doctors to see if the organs are being affected.

In the Republic of Ireland, most patients aged under 16 receiving chemotherapy for osteosarcoma will go to Our Ladys Hospital, Crumlin, Dublin. Data covering 2000-2010 showed that 37 paediatric osteosarcoma patients were treated there.

Data from 2004-2008, showed that patients aged 15-19, were seen at Mater Misercordiae Hospital, Our Lady's Hospital, Crumlin and Waterford Regional Hospital.

Patients aged over 20, were seen at Mater Misercordiae Hospital, Our Lady's Hospital Crumlin, Sligo General Hospital, Cork University Hospital, Waterford Regional Hospital, St Vincent's Hospital and Mercy Hospital.

Most patients (all ages) having surgery will go to St. Marys Orthopaedic, Cappagh.

Most patients (all ages) receiving radiotherapy will attend St Luke's and St Anne's Hospital, Dublin. However, some patients may also attend other hospitals in Dublin and Cork.

Figure 4. Bone Cancer Centres and Children and Young Adult Treatment Centres in the UK and Ireland.

Key

RedBone Cancer Centres
BlueChildren's Specialist Cancer Treatment Centre
GreenRegional Teenage/Young People's Cancer Centre
PurpleDedicated unit for teenage and young adult cancer
OrangeYoung Adult Oncology Service
BlackPrimarily Young Adults

Bone Cancer Treatment Centres:

North of England Bone and Soft Tissue Tumour Service, Newcastle upon Tyne Hospitals NHS Foundation Trust,
Nuffield Orthopaedic Centre NHS Trust, Oxford
Royal National Orthopaedic Hospital, Stanmore, Middlesex,
The Robert Jones and Agnes Hunt Orthopaedic and District Hospital NHS Trust, Oswestry,
The Royal Orthopaedic Hospital, Bristol Road South, Northfield, Birmingham,

Children's and Young Adults Specialist Cancer Treatment Centres:

Addenbrookes Hospital, Cambridge,
Alder Hey Hospital, Liverpool,
Beatson West of Scotland Cancer Centre, Glasgow,
Belfast City Hospital NHS Trust, Lisburn Road, Belfast (primarily young adults)
Birmingham Children's Hospital,
Bristol Royal Hospital for Children,
Christie Hospital, Manchester, (Dedicated unit for teenage and young adult cancer)
Great North Children's Hospital, Newcastle Upon Tyne
Leeds Teaching Hospitals NHS Trust (St James Hospital for adults; Leeds General Infirmary for children and teenagers)
Leicester Royal Infirmary (Young Adult Oncology Service),
Our Ladys Hospital, Crumlin, Dublin,
Queen Elizabeth Hospital, Birmingham,
Royal Aberdeen Children's Hospital,
Royal Belfast Hospital for Sick Children, Belfast,
Royal Hospital for Sick Children, Edinburgh
Royal Manchester Children's Hospital,
Southampton General Hospital,
St Luke's and St Anne's Hospital, Dublin,
St. Marys Orthopaedic, Cork,
The Royal Marsden Hospital, London
The University College Hospital, London,
Yorkhill Royal Hospital for Sick Children, Glasgow.

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 'Multidisciplinary Team' (MDT). The Multidisciplinary Team will work out the details of the treatment needed.

The MDT at the investigation (tests) and diagnosis stage will consist of:

  • Specialist bone sarcoma surgeons,

  • Specialist sarcoma oncologists (oncologists are doctors that look after people with cancer),

  • Specialist sarcoma pathologist (pathologists 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.

Patients that are having treatment for osteosarcoma will be under the care of the treatment and rehabilitation part of the multidisciplinary team including:

  • Specialist bone sarcoma surgeons,

  • Specialist sarcoma oncologists (oncologists are doctors that look after people with cancer),

  • Specialist nurses

  • Physiotherapists and occupational therapists will help with rehabilitation (rehab) after surgery.

  • Dieticians

  • Social workers and psychologists will help with patients' emotional, social and educational needs.

You can also read patients' and their family's experiences in our Stories by Patients and Families section.


Last reviewed: October 2010; Version: 1.1
Review due: October 2011

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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