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.

For a downloadable source of this information, please view our 'Downloadable Information Materials' page to view all of our fact sheets.

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.

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 aggressive yet very rare cancerous tumours that are able to present in any area of the body - usually in the soft tissues. They are most commonly reported in the breasts, heart, skin, liver and bones(1).

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 first step in diagnosing any primary bone cancer is usually a trip to the GP. Diagnosis of a suspected bone tumour usually follows a clinical examination and an X-ray. It is very common to be referred to a bone cancer specialist for a second opinion and confirmation of the diagnosis.

Further tests to confirm the presence of angiosarcoma of the bone include: CT scans, MRI scans, a biopsy of the bone and blood tests.

More information on these tests, such as CT scans, MRI scans and bone biopsies can be found here.

A CT scan and MRI scan are often non-specific and cannot definitively diagnose an angiosarcoma of the bone when used on their own. However, these tests do provide important information about the exact location of the tumour, the stage of the tumour and evidence of any spread of angiosarcoma to other areas of 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(2,3).

Angiosarcomas of the bone present with non-specific features when looked at by CT or MRI scans, which can make the diagnosis of this tumour type very difficult. However, it is known that an X-Ray can be more useful when identifying the presence of angiosarcoma than other imaging scans. If an angiosarcoma of the bone is presenting, the X-Ray will show an aggressive appearance, signifying the need for further tests.

Taking a biopsy of the bone is needed to confirm the diagnosis of an angiosarcoma of the bone(2). This specialist procedure takes a small sample of the tumour so it can be examined by a pathologist. This includes looking at the sample under a microscope and doing special tests for certain bone matrix proteins and molecules that are specific to this tumour type.

Results from a biopsy can take up to two weeks to become available and they enable doctors to confirm the presence of an angiosarcoma of the bone.

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).

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 recommended4. 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...