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.