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Primary Bone Cancer

bone cancer

Bone is a living tissue. The hard bone tissue is made of collagen fibres (tough, elastic fibres) and minerals (gritty, hard material). There are two main types of cells within the hard bone tissue that make and mould bone. One type (osteoblasts) makes and lays down bone material. Another type (osteoclasts) resorbs (dissolves) particles of bone.

In the centre of some larger bones is the soft bone marrow. This is where blood cells are made (red blood cells, white blood cells, and platelets).

What is bone cancer?

Bone cancer can be divided into primary bone cancer and secondary bone cancer.

Primary bone cancer means that the cancer started from cells in the hard bone tissue. It is a rare type of cancer. It affects males more frequently than females.

Secondary (metastatic) bone cancer means that a cancer which started in another part of the body has spread to a bone. Many types of cancer can spread to the bone. Most commonly, cancers of the breast, prostate, lung, kidney and thyroid. Secondary bone cancer is common. The behaviour, treatment and outlook of secondary bone cancers is often quite different to primary bone cancer.

Types of primary bone cancer

There are different types of primary bone cancer. They are classified by the type of cell which occurs in the cancer.

Osteosarcoma

This is the most common type of primary bone cancer, but even this is rare. It arises from bone-forming cells. Most cases occur in young people between the ages of 10 to 25, but it can occur at any age. It typically develops in the growing ends of the bone in young people, most commonly in bones next to the knee and the upper arms.

Ewing's sarcoma

Most cases occur in young people between the ages of 10 to 20, but it can occur at any age. It most commonly affects the pelvis (hips) and long bones in the leg.

Chondrosarcoma

This type of cancer arises from cartilage-forming cells. As well as occurring in the cartilage, a chondrosarcoma may also develop within a bone, or on the surface of a bone. Most cases occur in people between the ages of 40 and 75. It most commonly affects the pelvis, scapula (shoulder blade), ribs, and the bones of the upper parts of the arms and legs.

Other

Other rare types of primary bone tumor include fibrosarcoma, leiomyosarcoma, malignant fibrous histiocytoma, angiosarcoma and chordoma.

What causes primary bone cancer?

  • A cancerous tumour starts from one abnormal cell. The reason why a cell becomes cancerous is unclear. The most common types, osteosarcoma and Ewing's sarcoma, mainly occur in young people.
  • Your risk of developing a primary bone tumour is increased if you have:
  • High-dose radiotherapy to treat other problems.
  • Paget's disease of bone.
  • An osteochondroma (chondroma). This is a benign (non-cancerous) bone tumour which occasionally turns into a chondrosarcoma.
  • Certain rare inherited disorders including: Li-Fraumeni syndrome, hereditary multiple exostoses (HME), and hereditary retinoblastoma.
  • Ollier's disease (enchondromatosis) - a rare bone condition.

There is no evidence that having a previous trauma to a bone increases your risk of developing bone cancer in the future.

What are the symptoms of primary bone cancer?

  • Pain.
  • Swelling over the affected part of the bone.
  • Difficulty in moving a joint if the cancer is near to a joint.
  • Pressure symptoms if the tumour grows from the bone and presses on nearby structures. For example, pressure on a nerve may cause pain, tingling, weakness of muscles, or numbness of an area of skin.
  • A break (fracture) of a bone may occur at the site of the tumour after a minor injury.

General symptoms may occur as the cancer becomes larger - tiredness, weight loss, sweats. If the cancer spreads to other parts of the body, various other symptoms can develop.

How is primary bone cancer diagnosed and assessed?

If a doctor suspects that you may have primary bone cancer, you are likely to have a number of tests. These include  the following:

  • An X-ray. Primary bone cancers often have a characteristic appearance on an X-ray.
  • A bone scan. A bone scan involves an injection of a a small dose of radioactive material. This is taken up by active bone tissue. Cancerous bone tissue is very active and shows on a scanner as a 'hot spot'.
  • MRI scan. This is useful to show the exact site and size of a tumour.
  • A bone biopsy.

If you are confirmed to have primary bone cancer then further tests are usually advised to assess if the cancer has spread. This may include various blood tests, X-rays and scans.

Assessing the severity of the cancer - grading and staging

The results of the biopsy can show the features of the cells, and the cancer can be graded. For primary bone cancers, two grades are used:

  • Low-grade - the cells look reasonably similar to normal bone cells. The cancer cells are said to be 'well differentiated'. The cancer cells tend to grow and multiply quite slowly and are not so 'aggressive'.
  • High-grade - the cells look very abnormal and are said to be 'poorly differentiated'. The cancer cells tend to grow and multiply quite quickly and are more 'aggressive' and are more likely to spread.

The stage of primary bone cancer is based on the grade of the cancer, and how much it has grown or spread. The staging system commonly used is:

What are the treatment options for primary bone cancer?

The main treatments used for primary bone cancer are surgery, chemotherapy and radiotherapy. The treatment or combination of treatments advised in each case depends on various factors such as:

  • The type of primary bone cancer.
  • The exact site of the cancer.
  • The stage of the cancer (how large the cancer is and whether it has spread).
  • Your general health.

In some cases, treatment aims to cure the cancer. Doctors tend to use the word 'remission' rather than the word 'cured'. Remission means there is no evidence of cancer following treatment. If you are 'in remission', you may be cured.

In some cases, treatment aims to control the cancer. If a cure is not realistic, with treatment it may be possible to limit the growth or spread of the cancer so that it progresses less rapidly. This may keep you free of symptoms for some time.

In some cases, the aim of treatment is to ease symptoms only. This is called palliative treatment. For example, if a cancer is advanced then you may require painkillers or other treatments to help keep you free of pain or other symptoms.

Surgery

The types of operation vary depending on the type and site of the cancer. If the cancer is in an arm or leg, it is often possible to remove it with 'limb-sparing' surgery. However, amputation is still needed in some cases, depending on the size, spread or site of the tumor. Your specialist will advise on whether surgery is possible, and the types of operation which can be done.

Radiotherapy

Radiotherapy is a treatment which uses high-energy beams of radiation which are focused on cancerous tissue. This kills cancer cells, or stops cancer cells from multiplying.
Radiotherapy is usually used in combination with surgery and chemotherapy. Radiotherapy is not usually used for osteosarcomas or chondrosarcoma, as they are not very sensitive to radiation.

Chemotherapy

Chemotherapy is a treatment which uses anti-cancer medicines to kill cancer cells, or to stop them from multiplying. Chemotherapy may also be given after surgery or radiotherapy. This aims to kill any cancer cells which may have been left in the body.

What is the outlook?

It is difficult to give an overall outlook. Every case is different, and the success of treatment depends on the type, site and stage of the cancer - the earlier the stage, the better the outlook. In general, the outlook for primary bone tumors has improved in the last 10-20 years. This is due to improved surgical techniques and improved chemotherapy.


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