Bone Tumor

What is Bone Tumor?

Bone tumors develop when cells within a bone divide uncontrollably, forming a lump or mass of abnormal tissue. Most bone tumors are not cancerous (benign). Benign tumors are usually not life-threatening and, in most cases, will not spread to other parts of the body. Bone cancer destroys normal bone tissue. It may start in the bone or spread to other parts of the body (called metastasis).

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Here, you will find information about signs and symptoms, causes and risk factors, types, diagnosis, staging and prevention about bone tumor.

Symptoms of bone tumor are:

1

Pain: Pain in the affected bone is the most common sign of bone cancer. At first, the pain is not constant. It may be worse at night or when the bone is used, for instance, leg pain when walking. As the cancer grows, the pain will be there all the time, and get worse with activity.

2

Swelling: Swelling in the area of the pain may not occur until weeks later. It might be possible to feel a lump or mass depending on where the tumor is and can lead to trouble swallowing or make it hard to breathe.

3

Fractures: Bone tumor can weaken the bone it's in, but most of the time the bones do not fracture (break).

4

Unintentional weight loss and fatigue

5

Other symptoms: Cancer in the bones of the spine can press on nerves, causing numbness and tingling or even weakness.

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Some of the risk factors that develops bone cancer are:

1

Genetic disorders: A very small number of bone cancers (especially osteosarcomas) appear to be hereditary and are caused by defects (mutations) in certain genes. Retinoblastoma is a rare eye cancer in children that can be hereditary. The inherited form of retinoblastoma is caused by a mutation (abnormal copy) of the RB1 gene. Those with this mutation also have an increased risk of developing bone or soft tissue sarcomas. Also, if radiation therapy is used to treat the retinoblastoma, the risk of osteosarcoma in the bones around the eye is even higher.

2

Paget disease: Paget disease is a benign (non-cancerous) but pre-cancerous condition that affects one or more bones. It results in formation of abnormal bone tissue and occurs mostly in older people. Affected bones are heavy, thick, and brittle. They are weaker than normal bones and more likely to fracture (break). Most of the time, Bone cancer (usually osteosarcoma) develops in about 1% of those with Paget disease, usually when many bones are affected.

3

Radiation: Bones that have been exposed to ionizing radiation may also have a higher risk of developing bone cancer. A typical x-ray of a bone is not dangerous, but exposure to large doses of radiation does pose a risk. For example, radiation therapy to treat cancer can cause a new cancer to develop in one of the bones in the treatment area. Being treated when you are younger and/or being treated with higher doses of radiation increases your risk of developing bone tumor/cancer.

4

Exposure to radioactive materials: Such as radium and strontium can also cause bone cancer because these minerals build up in bones.

5

Bone marrow transplantation: Osteosarcoma has been reported in a few patients who have undergone bone marrow (stem cell) transplantation.

Types of bone tumors:

1

Bone tumors that are not cancer: Some tumors that start in the bone are benign (not cancer). Benign tumors do not spread to other tissues and organs and are not usually life threatening. They often can be cured with surgery. Types of benign bone tumors include: Osteoid osteoma, Osteoblastoma, Osteochondroma, Enchondroma, Chondromyxoid fibroma.

2

Malignant bone tumors: There are many different kinds of primary bone cancer/tumor. They're named based on the part of the bone or nearby tissue that's affected and the kind of cells forming the tumor.

1. Osteosarcoma: Osteosarcoma (also called osteogenic sarcoma) is the most common primary bone cancer. It starts in the bone cells. These tumors develop most often in bones of the arms, legs, or pelvis.
2. Chondrosarcoma: Chondrosarcoma starts in cartilage cells. It's the second most common primary bone cancer. Chondrosarcomas can start anywhere there's cartilage. Most develop in bones like the pelvis, legs, or arms. Sometimes chondrosarcoma starts in the trachea, larynx, or chest wall. Other sites are the scapula (shoulder blade), ribs, or skull.
3. Ewing tumor: Ewing tumor (also called Ewing sarcoma) is the third most common primary bone cancer. Most Ewing tumors develop in bones, but they can start in other tissues and organs. The most common sites for this cancer are the pelvis, the chest wall (such as the ribs or shoulder blades), and the long bones of the legs or arms.
4. Malignant fibrous histiocytoma: Malignant fibrous histiocytoma (MFH) most often starts in soft tissue (connective tissues such as ligaments, tendons, fat, and muscle); it's rare in bones. This cancer is also known as pleomorphic undifferentiated sarcoma, especially when it starts in soft tissues. When MFH occurs in bones, it usually affects the legs (often around the knees) or arms. MFH mostly tends to grow locally, but it can spread to distant sites, like the lungs.
5. Fibrosarcoma: This is another type of cancer that develops more often in soft tissues than it does in bones. Bones in the legs, arms, and jaw are most often affected.
6. Giant cell tumor of bone: This type of primary bone tumor has benign (not cancer) and malignant forms. The benign form is most common. Giant cell bone tumors typically affect the legs (usually near the knees) or arms. They don't often spread to distant sites, but after surgery tend to come back where they started. (This is called local recurrence.) This can happen many times. With each recurrence, the tumor becomes more likely to spread to other parts of the body. Rarely, a malignant giant cell bone tumor spreads to other parts of the body without first recurring locally.
7. Chordoma: This primary tumor of bone usually occurs in the base of the skull and bones of the spine. Chordomas tend to grow slowly and often do not spread to other parts of the body. They often come back in the same area if they are not removed completely. The lymph nodes, the lungs, and the liver are the most common areas for tumor spread.

3

Other cancers that develop in bones: Other cancers can be found in the bones, but they don't start in the actual bone cells. They are not treated like primary bone cancer.

1. Non-Hodgkin lymphomas: Non-Hodgkin lymphoma generally develops in lymph nodes but sometimes starts in the bone. Primary non-Hodgkin lymphoma of the bone is often a widespread disease because many bones are usually involved.
2. Multiple myelomas: Multiple myeloma almost always develops in bones, but it's not a primary bone cancer because it starts in the plasma cells of the bone marrow (the soft inner part of some bones).

An individual may need the following diagnostic tests:

1

Radionuclide bone scan: This can show whether cancer has spread to other bones. It can identify smaller areas of metastasized cancer than X-rays.

2

CT scans: These can indicate whether the cancer has spread and how far.

3

MRI scan: This type of scan can provide an outline of the tumor.

4

PET scan: This can be useful for scanning the whole body for cancer.

5

X-rays: Bone cancer will show on most X-rays. A chest X-ray can also show if the cancer has spread to the lungs.

6

Biopsy: To grade and stage a tumor, as well as assessing whether it is benign or malignant, a biopsy test is needed.

7

Blood tests: To determine the levels of two enzymes called alkaline phosphatase and lactate dehydrogenase. Large amounts of these enzymes may be present in the blood of people with osteosarcoma or Ewing sarcoma.

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After someone is diagnosed with bone cancer, doctors will try to figure out if it has spread, and if so, how far. This process is called staging. The stage of a cancer describes how much cancer is in the body. It helps determine how serious the cancer is and how best to treat it.

The staging system most often used for bone cancer is TNM system, which is based on 3 key pieces of information:
The extent (size) of the tumor (T): How large is the cancer? Is it in more than one spot in the bone?
The spread to nearby lymph nodes (N): Has the tumor spread to nearby lymph nodes?
The spread (metastasis) to distant sites (M): Has the tumor spread to the lungs only or to distant sites such as other bones or the liver?

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If you're interested in preventing bone tumor, take comfort in the fact that simple lifestyle changes can make a difference.

1

Don't use tobacco: Using any type of tobacco puts you on a collision course with cancer. Smoking has been linked to various types of cancer.

2

Eat a healthy diet: Having healthy diet might reduce your risk of getting bone cancer.

3

Maintain a healthy weight and be physically active: Maintaining a healthy weight might lower the risk of bone tumor and various types of cancer.

4

Physical activity: In addition to helping you control your weight, physical activity on its own might lower the risk of bone tumor/cancer.

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How Bone Tumor is treated?

The type of treatment for bone cancer depends on several factors, including: the type of bone cancer, its location in the body, how aggressive it is, whether it has spread and as well as the person's age and general health. Treatment options for bone cancer include surgery, chemotherapy, radiation therapy, cryosurgery, and targeted therapy.

Surgery is the primary (main) treatment for most kinds of bone tumor. The main goal of surgery is to remove all of the tumor. If even a few cancer cells are left behind, they can grow and make a new tumor. To try to be sure that this doesn't happen, surgeons remove the tumor plus some of the normal tissue around it. This is called wide-excision. Taking out some normal tissue helps ensure that all of the cancer is removed.

1. Tumors in the arms or legs:
Amputation: Amputation is surgery to remove part or all of a limb (an arm or leg). When used to treat cancer, amputation removes the part of the limb with the tumor, some healthy tissue above it, and everything below it.

Limb-salvage surgery: The goal of limb-salvage surgery is to remove all of the cancer and still leave a working leg or arm. Most patients with bone cancer in a limb are able to have their limb spared. This type of surgery is very complex and requires surgeons with special skills and experience. In limb-salvage surgery, a wide-excision is done to remove the tumor. A bone graft or an endoprosthesis (meaning internal prosthesis) is used to replace the bone that's lost.

Reconstructive surgery: After amputation, surgery can be done to rebuild or reconstruct a new limb.

2. Tumors in other areas:
Bone tumor in the pelvis is treated with a wide-excision when possible. If needed, bone grafts can be used to rebuild the pelvic bones.

For a tumor in the lower jaw bone, the entire lower half of the jaw may be removed and later replaced with bones from other parts of the body.

For tumors in areas like the spine or the skull, it may not be possible to safely do a wide-excision. Cancers in these bones may require a combination of treatments such as curettage, cryosurgery, and radiation.

Bone cement: The bone cement PMMA (polymethylmethyacrylate) starts out as a liquid and hardens over time. It's put into the hole in the bone in liquid form. As it hardens, it gives off a lot of heat. The heat helps kill any remaining tumor cells. This allows PMMA to be used without cryosurgery for some types of bone tumors.

3. Surgical treatment of metastasis:
To be able to cure a bone cancer, it and any existing metastases must be removed completely with surgery. The lungs are the most common site of distant spread for bone cancer. Surgery to remove bone cancer metastases to the lungs must be planned very carefully. Before the operation, the surgeon will consider the number of tumors, where they are (in one lung or both lungs), their size, and the person's overall health.

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Radiation therapy uses high-energy rays or particles to kill tumor cells. External beam radiation therapy is radiation delivered from outside the body that's focused on the tumor. This is the type of radiation therapy used to treat bone tumor or bone cancer.

Most bone tumors are not easily killed by radiation, and high doses are needed. High doses, however, can damage nearby healthy tissues, as well as key structures (like nerves and blood vessels) in the area.

Most of the time, radiation is used to treat bone cancers that are unresectable. This means they cannot be completely removed with surgery. Radiation may also be used after surgery if cancer cells were found in the edges (margin) of the removed tissue. Another term for this is positive margins. In this case, radiation may be given to kill any cancer that may have been left behind.

If bone cancer comes back after treatment, radiation can help control symptoms like pain and swelling.

Types of radiation therapy: Special types of radiation are most commonly used to treat bone cancer. These types give the best control over the size and strength of the radiation beams so that higher doses get to the tumor and spare the nearby tissues.

1. Intensity-modulated radiation therapy: Intensity-modulated radiation therapy (IMRT) is an advanced form of external beam radiation therapy. With this technique, a computer matches the radiation beams to the shape of the tumor and can adjust the intensity (strength) of the beams. The radiation is delivered to the tumor from many directions to reduce the amount of radiation that goes through any one area of normal tissue. This makes it possible to reduce radiation damage to normal tissues while increasing the radiation dose to the tumor.

2. Proton-beam radiation: Proton-beam radiation is a special form of radiation that uses protons instead of regular x-rays to kill cancer cells. They cause little damage to the tissues they pass through but are very good at killing cells at the end of their path. This allows a high dose of radiation to be given to the tumor without hurting the normal tissue around it. This form of radiation is very helpful in treating skull, spine, rib, or sternum (breast bone) chondrosarcomas and chordomas.

3. Extracorporeal radiation: Though not commonly used, another option is extracorporeal radiation. This may be used when trying to save a limb as part of limb-salvage surgery. It involves taking the bone with tumor out of the body, treating it with radiation, and then putting it back in.

Chemotherapy (chemo) is the use of drugs to treat bone tumor. Chemo is systemic treatment. This means that the drugs go into the bloodstream and circulate to reach and destroy bone tumor cells all over the body.

Chemo is often a part of treatment for Ewing sarcoma and osteosarcoma. It can be useful for a certain type of chondrosarcoma called mesenchymal and high-grade dedifferentiated chordomas. It may be used along with targeted therapy for some giant cell tumors. Chemo is sometimes used for bone cancer that has spread through the bloodstream to the lungs and/or other organs.

The drugs mainly used to treat bone tumor/cancer include: Doxorubicin (Adriamycin®), Cisplatin, Etoposide (VP-16), Ifosfamide (Ifex®), Cyclophosphamide (Cytoxan®), Methotrexate, Vincristine (Oncovin®). In most cases, several drugs (2 or 3) are given together.

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Targeted therapy drugs, work differently from standard chemotherapy (chemo) drugs and are especially important in diseases like chordomas and other bone cancers, where chemo has not been very useful. They may prove to be a better treatment for these cancers.

Imatinib: Some chordomas have gene defects (mutations) that make proteins that signal the cells to grow. The drug imatinib (Gleevec®) is a targeted therapy drug that can block the signals from these genes. This can make some tumors stop growing or even shrink a little. Imatinib is used to treat chordomas that have spread or have come back after treatment.

Denosumab: Denosumab (Xgeva® or Prolia®) is a monoclonal antibody (a man-made version of an immune system protein) that binds to a protein called RANK ligand. RANK ligand normally tells cells called osteoclasts to break down bone, but when denosumab binds to it, that action is blocked. In patients with giant cell tumors of bone that have either come back after surgery or cannot be removed with surgery, denosumab can help shrink tumors for a while.

Interferon: Interferons are not exactly targeted therapies. They're a family of substances naturally made by our immune system. Interferon-alpha is the type that may be used to treat giant cell tumors of the bone that have come back after treatment (recurred) or spread (metastasized). This drug is most often given as a daily injection under the skin. It can also be injected into a muscle or vein.

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