Angiosarcoma of the Bone

This information has been written for patients, their families and friends and the general public to help them understand more about a rare form of primary bone cancer, known as angiosarcoma. This section will detail what an angiosarcoma of the bone is and how angiosarcomas are diagnosed and treated.

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What is angiosarcoma of the bone?

Angiosarcoma is a malignant (cancerous) tumour starting in cells which line the blood vessels. Therefore it can develop anywhere in the body – in any organ. They are most commonly reported in the breasts, heart, skin, liver and bones(1).

This section will focus on angiosarcomas of the bone, which are very rare and account for less than 1% of all primary bone cancers.

Angiosarcomas of the bone most commonly arise in the shinbone (known as the tibia), the thighbone (known as the femur) or the hip bone (known as the pelvis).

Angiosarcomas are able to arise in various locations in the body because they develop in cells known as endothelial cells, which form the lining of the blood vessels. When an endothelial cell of the blood vessel becomes abnormal and grows uncontrollably, an angiosarcoma may develop(2).

Angiosarcomas of the bone make up just 6% of all reported cases of angiosarcoma. When presenting in the bone, this tumour most commonly affects the long tubular bones and the axial skeleton; which is an area of the skeleton that includes the skull, spine and rib cage. The bones with the most reported cases of angiosarcoma are the thighbone (known as the femur), the shinbone (known as the tibia), the pelvis, the spine and the long bone of the arm (known as the humerus)(3).

Unfortunately, angiosarcomas of the bone can vary in the way they behave and can often grow quickly which can make these tumours challenging to diagnose. This rare type of primary bone cancer has a high risk of returning at a later date (known as tumour recurrence) and can often spread to other bones in nearby areas of the body. For this reason, it is important that the doctors are fully aware of the extent of an angiosarcoma of the bone before beginning treatment. This ensures a suitable treatment plan is made in advance, to allow patients to receive the best care possible.

Angiosarcomas of the Bone can affect anyone, at any age. Due to this cancer being rare and there being a low number of reported cases to review, it is difficult to confirm any trend in its occurrence.

Despite the low incidence, it is thought that this cancer presents slightly more often in males than females. Although angiosarcomas of the bone have been reported in patients between the ages of 18 and 82, it most frequently occurs between 20 to 50 years of age(2,3,4).

Angiosarcomas of the Bone often grow rapidly and can sometimes present as an incidental finding, with no symptoms at all in the early stages. When a disease presents without any symptoms it is known as ‘asymptomatic’.

When symptoms do present, they depend on the size of the tumour and the location of the tumour in the body. Pain and swelling, the most common symptoms, can often be present for months or even years before a diagnosis is made(2).

The most commonly reported symptoms of an angiosarcoma of the bone are:

Bone pain - this pain may be a dull ache or a stabbing sensation
Tiredness – also known as fatigue
Swelling or a lump
On rare occasions (around 10%) the tumour can cause the bone to fracture - known as a pathological fracture

If the tumour is located near the spine, as well causing back pain, the tumour can put pressure on the spinal cord or nerves, leading to neurological symptoms(2). These symptoms may include numbness or weakness when using the arms or legs and a sensation of pins and needles.

For more information on the symptoms of primary bone cancer please see our About Primary Bone Cancer information section.

Although there is only one known type of Angiosarcoma of the Bone, this tumour can present in different ways and these variations may require different treatment plans.

Angiosarcomas may present as:

A single lesion
Multiple lesions in a single bone
Multiple lesions in multiple bones

A lesion refers to an area that is involved with the tumour.

The most common presentation is that of multiple lesions of the tumour in the same bone, followed by multiple lesions presenting in bones that are nearby to one another(2,4).

Much like many types of primary bone cancer, the cause of an angiosarcoma of the bone is unknown and studies of the causes are difficult because the tumour is rare(2).

Although there is currently no identifiable cause, some conditions may increase the likelihood of an individual developing an angiosarcoma of the bone.

These include:

Bone Infarction

Bone infarction is the death of an area of bone because of a lack of blood supply and therefore oxygen to the area. This occurs most frequently around the knee

Paget’s Disease of the Bone

One of the most common diseases of the bone is Paget’s disease, this causes weakening and pain in areas of bone. For more information on Paget’s disease of the bone please visit www.paget.org.uk

Chronic Osteomyelitis

Chronic osteomyelitis is an infection of the bone, usually caused by bacteria. This can be difficult to eradicate and it commonly returns after treatment.

Patients with different types of primary bone cancer are assessed in similar ways. For this reason, diagnostic tests are covered in more detail in our About Primary Bone Cancer information section.

This section aims to provide information on the specific details of diagnosing an angiosarcoma of the bone and discuss other conditions that may appear diagnostically similar to this form of angiosarcoma.

The symptoms of angiosarcoma can be general. There is no one clear sign that doctors can easily look for to make a diagnosis of angiosarcoma.

People report a variety of experiences when they seek medical advice about their symptoms. Most people with worrying symptoms go to their GP.

Some patients go to their local hospital emergency department (A&E) or other health care centres.

Some people are referred quickly for further tests or a second opinion, but often patients have to return to their GP three or four times before they are referred for more tests. Primary bone cancers are very rare and many GPs will never come across a case.

If a GP or hospital doctor is concerned about the patient's symptoms, there are national guidelines they should follow. According to the National Institute for Clinical Excellence (NICE) Guidelines for Suspected Bone Cancer and Sarcoma:

Children, teenagers and young adults with unexplained bone swelling or bone pain should have an urgent X-ray within 48 hours. If the X-ray suggests a possible bone cancer, your GP should refer you to a specialist within 48 hours.
Adults should be seen by a specialist within 2 weeks if the results of an X-ray suggest a bone cancer.

If the X-ray is normal but symptoms persist, the patient should be followed up and/or a repeat X-ray or MRI scan should be carried out within 2 weeks (adult) or within 48 hours (child) or a referral requested to a specialist.

Bone Cancer Awareness Initiative

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. Our 2020 Patient Survey report is the most comprehensive analysis of presenting symptoms and routes to diagnosis for primary bone cancers & tumours in the UK to date. This is our evidence base on which we will focus our awareness objectives moving forward.

The report focuses on two main areas - the time and routes to diagnosis and the range of presenting symptoms across all anatomical locations and forms of primary bone cancer & bone tumours.

Our analysis found that patients wait, on average, more than 7 months and make 8 visits to the multiple healthcare professionals before receiving an accurate diagnosis.

Going to a Bone Cancer Centre for more tests

Once an abnormality is found in a bone that suggests the possibility of cancer, the patient will be referred to a bone sarcoma surgical centre.

Bone sarcoma surgical centres are specialist hospitals. They have a group of healthcare specialists who are experts in the diagnosis and management of bone cancer.

In England, there are currently five bone sarcoma centres which specialise in the diagnosis and management of primary bone cancers. These centres are at Birmingham, Newcastle, Oswestry, Oxford, and Stanmore.

In the Republic of Ireland, there are no specific bone sarcoma 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.

Patients in Wales 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.

For a full list of locations patients may be referred to in order to confirm a primary bone cancer diagnosis please click here

The MDT

Specialists in many different areas of medicine at the bone cancer centres, the Regional Cancer Centres in the UK and hospitals in Ireland work together as a 'Multidisciplinary Team' (MDT). The members of the MDT work together to diagnose the patient's condition.

The MDT includes:

Specialist sarcoma surgeons.
Specialist sarcoma oncologists (oncologists are doctors who look after people with cancer).
Specialist sarcoma pathologists (pathologists are doctors who use laboratory techniques to diagnose disease).
Radiologists (doctors who diagnose disease and conditions from looking at x-rays, or scans).
Sarcoma cancer nursing specialists (sometimes called 'CNS') who perform an essential role in treating and caring for primary bone cancer patients.

What tests are done?

When a person is referred to a bone cancer surgical centre, further tests will be done to find out more and to confirm whether the patient has bone cancer, and if so what type.

These tests may include:

X-ray

X-rays of the bone may be taken, including the joints above and below, and 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.Cancer Research UK gives more information about X-rays.

Blood tests

These include:

- Blood chemistry (Urea and Electrolytes) 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
- Full blood count (FBC) counts the numbers of different types of blood cells in the patient's blood at that time.
- Red blood cells - which carry oxygen in the blood.
- White blood cells - which are essential to the immune system (and totals of each type).
- Platelets - which are essential to the making blood clots and scabs.
- Levels of haemoglobin - which is found in red blood cells.
- Liver function tests (LFTs) to see how the liver is working.
- Erythrocyte Sedimentation Rate (ESR) is a test to look for signs of inflammation.
- C-Reactive Protein (CRP) levels are tested as CRP levels increase in inflammation.
- Alkaline phosphatase (ALP) levels are measured in patients with suspected osteosarcoma.

Cancer Research UK gives more information about blood tests.

MRI scan

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 scan of the entire bone is used to gain more information about the tumour in the bone. An injection of a special dye, known as a contrast agent is also used. 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. For some patients they may also have a total body MRI scan to look for areas of abnormalities in the other bones such as tumour spread (metastases).

Cancer Research UK gives more information about MRI scans.

CT scan

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 cross-sections of the inside of the body.

CT scanning of the lungs shows up any secondary tumours where the cancer may have spread (metastases). It is used if the MRI scan results have not been able to confirm the diagnosis of osteosarcoma.

Cancer Research UK gives more information about CT scans.

A CT scan and MRI scan cannot definitively diagnose an adamantinoma. However, they provide important information on the exact location of the tumour, the stage of the tumour and the presence of the tumour having spread anywhere else in the body. Additionally, these scans are useful for planning the treatment of individual patients and deciding on the best way to surgically remove the tumour(2).

PET scan

PET stands for 'positron emission tomography.' Not all hospitals have PET scanners but they are used to detect spread or metastases in osteosarcoma.

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 fluorine18 will be given.

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. Hot spots on the PET scan can detect metastases.

The results of the scan will be examined by a radiologist.

Cancer Research UK gives more information about PET scans.

Biopsy

Taking a biopsy of the bone is needed to confirm the diagnosis of adamantinoma. A bone biopsy is a specialised procedure that can be performed by a specialist in orthopaedic surgery or sarcoma radiology at a bone cancer surgical centre. 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 being taken may be a ‘needle biopsy’ or an ‘open biopsy’.

Needle biopsy: a needle is inserted into the tumour to draw out a small amount of tumour tissue (this may be done under local anaesthetic). Often, in order to know exactly where to take the sample from, this test is carried out alongside an X-ray or CT scan to guide the doctor.
Open biopsy (or surgical biopsy): is used less frequently than a needle biopsy. This form of biopsy is carried out during a small, minor, operation to remove a small piece of tumour while under general anaesthetic. This test tends to be used if a needle biopsy does not provide a diagnosis and the doctor wish to investigate further.

Cancer Research UK gives more information about bone biopsies.

An alternative diagnosis?

There are various conditions and other tumours that present in a similar manner to an angiosarcoma of the bone. These similarities may be in the patients’ symptoms, in the appearance of the tumour under the microscope, or in the appearance of a scan (such as an X-Ray, CT scan or MRI scan)(2). It can sometimes take a long time to confirm a diagnosis of primary bone cancer after a biopsy, this is because these tumours are rare and sometimes difficult to identify. Conditions which can be the cause of particular symptoms are known as ‘differential diagnoses’.

Other conditions which can present in the same way as an angiosarcoma of the bone include:

Metastatic Carcinoma

When a cancer spreads from one area of the body to form a tumour in another area of the body, the secondary tumour is known as a metastatic carcinoma. Many cancers spread to the bone, including those from the breast, lung and kidney, and so an angiosarcoma of the bone is often thought to be a metastatic carcinoma

Epithelioid Hemangioendothelioma:

Epithelioid hemangioendothelioma is a rare tumour that arises in the bone, liver and the lungs(6).

If a diagnosis of angiosarcoma of the bone is confirmed, you will need treatment in a bone cancer centre.

For more information on the locations of Bone Cancer Centres please click here.

Due to the rarity of angiosarcoma, and particularly angiosarcoma of the bone, it is difficult to develop new treatments and treatment often involves several members of the sarcoma team(1,3).

Where will treatment take place?

Surgery needs to take place at one of the bone cancer surgical centres (see map below, blue stars). Chemotherapy and radiotherapy can take place at different hospitals around the UK and Republic of Ireland. The delivery of intensive chemotherapy should be administered at one of the specialised cancer centres. For patients whose nearest specialist hospital is far away, a 'shared care' arrangement for acute issues and unexpected admissions with a closer hospital might be set up. The specialist hospital can be reached for advice on acute presentations and outpatient management.

England and Wales

Diagnosis and surgery should take place in one of the five bone cancer centres (see the map below):

Scotland

Patients are treated at one of the five Sarcoma Centres that are part of the Scottish Sarcoma Network. These hospitals are in Aberdeen, Dundee, Edinburgh, Glasgow, and Inverness. Patients visit one of these five Sarcoma Centres for chemotherapy or radiotherapy treatment. For surgery, primary bone cancer patients are seen in Glasgow, Edinburgh or Aberdeen.

Republic of Ireland

Most patients aged under 16 receiving chemotherapy fare treated at Our Lady's Hospital, Crumlin, Dublin.

Patients aged 15-19 are treated at Mater Misercordiae Hospital, Our Lady's Hospital, Crumlin and Waterford Regional Hospital.

Patients aged over 20 are treated 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.

For surgery, most patients in the Republic of Ireland (all ages) go to St. Marys Orthopaedic, Cappagh. For radiotherapy most patients attend St Luke's and St Anne's Hospital, Dublin. However, some patients may also attend other hospitals in Dublin and Cork.

Map of UK and Ireland showing location of specialist bone cancer treatment centres

Key

Red stars: Specialist Children's Cancer and Leukaemia Centre
Blue stars: Bone Cancer Surgical Centre
Green stars: Children and Young People's Integrated Cancer Service
Purple stars: Teenage Cancer Trust Unit
Yellow stars: Scottish Sarcoma Network Hospital

Please see our full list of centres providing treatment for primary bone cancer here.

Surgery

As with many types of primary bone cancer, the most effective treatment, if possible, is the surgical removal of the tumour. Surgery requires ‘wide surgical margins’; this means some healthy tissue is removed alongside the tumour to ensure all tumour cells are removed and there is a lower risk of the tumour returning at a later date.

Usually, limb-sparing surgical techniques are widely used, which means the tumour is completely removed while keeping as much of the normal function and cosmetic appearance of the limb as possible.

Common types of limb-sparing surgery performed to treat angiosarcoma of the bone are:

Autografts - once the tumour is removed the affected area of bone is reconstructed using the patients’ own tissue from another area of their body. The only downside to this surgical procedure is that the patient will have two surgical sites to recover from, which may create additional recovery time
Allografts - an allograft is a procedure using donated tissue to reconstruct the affected area of the bone once the tumour has been removed
Metallic Replacement - once the tumour is removed, the area of damaged bone is replaced with a metal implant known as a prosthesis. This procedure usually requires rehabilitation therapy after surgery, such as physiotherapy, to allow the patient to return to an active day-to-day life

Unfortunately, if the tumour has already spread to other areas of the body (a process known as metastasis) before a treatment plan is created, then surgery to the original tumour is not always beneficial and other treatments may be offered. In some cases, where the tumour can’t be completely removed or removal means that the limb would not be useful afterwards, amputation (the removal of the limb) may be recommended. Where possible, patients will be provided with a prosthesis (an artificial limb) made specifically for that individual.

Radiotherapy and Chemotherapy

Radiotherapy and chemotherapy are often used alongside surgery to treat angiosarcoma of the bone, or may be used as an alternative to surgery in some cases. Some patients may not receive either of these treatment methods, and some may receive both.

If the angiosarcoma of the bone is fast growing and more likely to spread elsewhere in the body, the patient is likely to receive chemotherapy before surgery is performed. This aims to shrink the tumour, allowing a more successful surgical procedure, and may help to treat any tumour cells that have already spread elsewhere in the body. Whether or not the treatment is continued will depend on whether or not the tumour has responded to chemotherapy, and the benefit of treatment is always balanced against the risks and side-effects.

Chemotherapy may also be used if the patient does not wish to undergo surgery or is not fit for surgery to take place.

Radiotherapy is most frequently used after surgery (known as adjuvant radiotherapy). The use of surgery and radiotherapy together provides the best possible outcome for patients with a single lesion of angiosarcoma of the bone(1,3). However, if the tumour has already spread to other areas of the bone, adjacent bones or other areas of the body, radiotherapy is of little use to the patient.

In some cases radiotherapy may be given to relieve the pain that patients may be experiencing – in these cases the treatment is known as ‘palliative radiotherapy’(4).

For more information regarding treatment procedures, including surgery, chemotherapy and radiotherapy, please visit our About Primary Bone Cancer information page.

Outcome

In order for each patient to receive the best possible treatment the true extent of the angiosarcoma of the bone needs to be determined before treatment can begin(3). This is so that an appropriate treatment plan can be developed after a discussion with the patient.

The outlook after a diagnosis of angiosarcoma of bone is variable and depends on the size of the tumour and whether it has spread elsewhere in the body. Spread of the angiosarcoma makes it much more difficult to cure the patient(3,4).

It is important to bear in mind that patients receiving treatment outside the UK may receive different tests and treatment plans in accordance to the treatment guidelines set out in that specific country. If you have any questions or concerns regarding this please discuss this with your medical team or contact The Bone Cancer Research Trust for more information.

If you would like more information about angiosarcoma of the bone please contact us.

Key References:

1. Patel, V.B, Speer, T.W. Successful Treatment of an Angiosarcoma of the Nose with Radiation Therapy. Case Reports in Oncology. 2012; 5(3): 570-575. Available at: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC350605...

2. Baliaka, A, Balis, GC, Michalopoulou-Manoloutsiou, E, Papanikolaou, A, Nikolaidou, A. Primary Angiosarcoma of Bone: A Case Report. Hippokratia. 2013; 17(2):180-182. Available at: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC374362...

3. Yasmashita, H, Endo, K, Teshima, R. Angiosarcoma of the Proximal Humerus: A Case Report and Review of the Literature. Journal of Medical Case Reports. 2012; 6:347. Available at: http://jmedicalcasereports.biomedcentral.com/artic...

4. Palmerini, E, Maki, R.G, Staals, E.L, Alberghini, M, Antonescu, C.R, Ferrari, C, Ruggieri, P, Mavrogenis, A, Bertoni, F, Cesari, M, Paioli, A, Marchesi, E, Picci, P, Ferrari, S. Primary Angiosarcoma of Bone: A Retrospective Analysis of 60 Patients From Two Institutions. American Journal of Clinical Oncology. 2014; 37(6): 528-534. Available at: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC368191...

5. Goldblum, J. R, Weiss, S.W, Folpe,A.L. Enzinger and Weiss’s Soft Tissue: Sixth Edition, 2014. Elsevier Saunders, Philadelphia, ISBN: 978-0-323-08834-3

6. Sardaro, A, Bardoscia, L, Petruzzelli, M, Portaluri, M. Epithelioid Hemangioendothelioma: An Overview and Update on a Rare Vascular Tumour. Oncology Reviews. 2014; 8(259): 82-91. Available at: http://www.oncologyreviews.org/index.php/or/articl...