Disease: Bone Cancer

What is bone cancer? What is metastatic bone cancer?

Bone cancer is a cancer of the cells that make up the bones of the body. When cancer is found in bones, it has usually started in another organ or another location in the body and has spread to the bones. This is known as metastatic cancer and is named for the site at which the original cancer started (for example, metastatic colon cancer) and is not referred to medically as bone cancer. It is much more common than true, or primary, bone cancer, where the bone cells themselves become malignant. Primary and metastatic bone cancers are often treated differently and may have a different prognosis.

There are other cancers that may begin in the bone even though they are not considered to be true bone cancers. Lymphoma is a cancer of the cells involved in the immune response. Lymphoma usually begins in the lymph nodes, but it sometimes begins in the bone marrow. Multiple myeloma is another cancer of the immune cells that typically begins in the bone marrow. These tumors are not considered to be primary bone cancers because they do not arise from bone cells.

This article focuses on primary bone cancer, cancer of the bone cells.

Who is at risk for bone cancer?

Each year, around 2,300 cases of cancer of the bones are diagnosed in the U.S. Primary bone cancers are not common and make up far less than 1% of all cancers. Bone cancers are more common in young adults and children than in older people. Cancer found in the bones of an older adult most likely is metastatic from another location in the body.

Risk factors have been identified for the development of certain bone cancers. Risk factors include the following:

  • Previous treatment with radiation therapy
  • Previous chemotherapy with drugs known as alkylating agents
  • Mutation in a gene known as the retinoblastoma (Rb) gene
  • Associated conditions, such as hereditary retinoblastoma, Paget's disease of bone, Li-Fraumeni syndrome, and Diamond-Blackfan anemia

What causes bone cancer?

As with other cancers, there is no one cause of bone cancer. In general, cancers arise when pathways that control normal cell growth and proliferation are disrupted, allowing abnormal cells to divide and grow uncontrollably. A number of hereditary and environmental factors are likely involved in the development of bone cancers.

What are bone cancer symptoms and signs?

Pain is the most common symptom of bone cancer. The pain may begin at certain times, often at night, or with physical activity and tends to worsen over time. Sometimes, the pain may be present for years before the affected person seeks treatment. Other cases of bone cancer are discovered incidentally when X-rays or imaging studies are performed for another reason. A mass, swelling, or lump is sometimes felt around the area of a bone cancer. Bone fractures may also occur at the site of the bone tumor (including benign tumors as well as cancers) because the underlying bone has weakened. Other less common symptoms can result from compression or disruption of nerves and blood vessels in the affected area. These symptoms may include numbness, tingling, tenderness, or a decrease in blood flow (coldness, weakened pulse).

What are the different types of bone cancer?

There are several different types of bone cancer that can affect different patient populations, and they are often treated differently. Knowing the precise type of bone cancer is important to develop a customized management and treatment plan for the specific patient. Some of the most common types of bone cancer are discussed in the following section.

Osteosarcoma

Osteosarcoma is the most common type of bone cancer. Osteosarcoma occurs most commonly in teenagers and young adults (ages 10-19) and is more common in males. Osteosarcoma in young people tends to develop at the ends of long bones in areas of active bone growth, often around the knee, either at the end of the femur (thighbone) or the proximal part of the tibia (shinbone). The next most common location is in the bone of the upper arm, but it is possible for an osteosarcoma to develop in any bone. Depending on the appearance of the tumor cells under the microscope, there are several different subtypes of osteosarcoma.

Chondrosarcoma

Chondrosarcoma is the second most common bone cancer. It arises from cartilage cells. It is more common in people older than 40 years of age. It may be either rapidly growing and aggressive or slow-growing. Chondrosarcoma is most commonly found in the bones of the hips and pelvis.

Ewing sarcoma

Ewing sarcomas, sometimes referred to as the Ewing sarcoma family of tumors (ESFTs), are an aggressive form of bone cancer that is most common in children 4-15 years of age. It can occur either in the bones or in the soft tissues and is believed to arise from primitive nerve tissue. ESFTs are more common in males than in females and are extremely rare in African-American children. The most common location for Ewing sarcoma is the middle portion of the long bones of the arms and legs.

Pleomorphic sarcoma (malignant fibrous histiocytoma) of bone

Pleomorphic sarcoma is a tumor formerly referred to as malignant fibrous histiocytoma or MFH. This term is still used frequently. Pleomorphic sarcomas are typically tumors of the soft tissues, but in up to 5% of cases they may arise in the bone. Pleomorphic sarcomas typically occur in adults and can be found anywhere in the body.

Fibrosarcoma

Fibrosarcoma is an uncommon type of bone cancer. It is most commonly arises in adults behind the knee.

What kinds of bone cancer occur in children?

As mentioned above, osteosarcoma, the most common type of bone cancer, is most common in children and young adults. Ewing sarcoma is another bone cancer that typically affects children.

What tests are used to diagnose bone cancer?

A variety of imaging tests may be used to identify bone tumors, including bone cancers. Very early bone cancers may not be apparent on plain X-rays, but X-ray examination can identify many tumors. CT scans are more precise and may be used to identify bone tumors. MRI scans can provide detail of the soft tissues of the body and can typically reveal whether the cancer has spread beyond the bone into nearby tissues.

A bone scan is a test that uses radioactive material and X-rays to identify areas of rapidly growing or remodeling bone. This may allow the localization of tumors anywhere in the body. This test is not specific for bone cancers and can also reveal areas of inflammation as found with arthritis, fractures, and infections.

While many bone cancers have a characteristic appearance on imaging studies, a biopsy (tissue sample) must be taken in order to precisely determine what kind of cancer is present and confirm the diagnosis. The biopsy can be taken with a needle or through a surgical incision.

What is the treatment for bone cancer?

Surgical removal of the tumor is the mainstay of treatment for bone cancers. Improved surgical techniques allow for most bone cancers to be removed without requiring amputation of an affected limb. However, reconstructive surgery is often done in addition to tumor removal in order to maximize function.

For most types of bone cancers, chemotherapy drugs are given in addition to surgery. One exception is chondrosarcoma, which is typically not treated with chemotherapy. Radiation therapy is often used for chondrosarcomas and for Ewing sarcomas.

Ewing sarcomas that do not respond well to other treatments are sometimes treated with high-dose chemotherapy followed by a stem cell transplant. In this procedure, the patient's stem cells (blood cells that have the potential to make all the different kinds of blood cells) are harvested from the bloodstream in a way similar to a blood donation. After high doses of chemotherapy medications to destroy the bone marrow, the harvested stem cells are returned to the body as with a blood transfusion. Over the next three to four weeks, the stem cells produce new blood cells from the bone marrow.

How is bone cancer pain managed?

The pain of bone cancer is medically treated with analgesic (pain-relieving medications). These may be nonprescription or prescription medications. Mild-to-moderate pain is treated with medications such as acetaminophen (Tylenol) or nonsteroidal anti-inflammatory medications (NSAIDs), including ibuprofen and naproxen. However, people taking anticancer chemotherapy drugs may need to avoid the use of NSAIDs due to the increased risk of bleeding.

Prescription medications may be used for moderate-to-severe cancer pain. Opioids -- stronger narcotic pain medications -- like codeine, morphine, oxycodone (Oxycontin), hydromorphone (Dilaudid), and fentanyl may be required to control severe pain. Sometimes a combination of medications is used to treat cancer pain. Opioid medications can be associated with side effects like drowsiness, constipation, and nausea.

What is the prognosis for bone cancer?

The prognosis, or outlook, for bone cancer depends upon the particular type of cancer and the extent to which it has spread. The overall five-year survival rate for all bone cancers in adults and children is about 70%. Chondrosarcomas in adults have an overall five-year survival rate of about 80%.

The five-year survival rate specifically for localized osteosarcomas is about 60%-80%. If metastases are present, meaning that the cancer has spread beyond bone, the survival rate is about 15%-30%, but there are factors that may affect the prognosis. Osteosarcomas tend to have a more favorable prognosis if they are located in an arm or leg, respond well to chemotherapy, and are able to be completely removed at surgery. Younger patients and females also tend to have a more favorable prognosis than males or older patients.

Ewing sarcomas have a five-year survival rate of about 70% when they are found in a localized stage. If they have spread to other sites (metastasized), the survival rate drops to 15%-30%. Certain factors have been associated with a more favorable prognosis for Ewing sarcomas, including smaller tumor size, patient age less than 10, having a tumor in an arm or leg (in contrast to a tumor in the pelvis or chest wall), and having a good response to chemotherapy drugs.

What causes bone cancer?

As with other cancers, there is no one cause of bone cancer. In general, cancers arise when pathways that control normal cell growth and proliferation are disrupted, allowing abnormal cells to divide and grow uncontrollably. A number of hereditary and environmental factors are likely involved in the development of bone cancers.

What are bone cancer symptoms and signs?

Pain is the most common symptom of bone cancer. The pain may begin at certain times, often at night, or with physical activity and tends to worsen over time. Sometimes, the pain may be present for years before the affected person seeks treatment. Other cases of bone cancer are discovered incidentally when X-rays or imaging studies are performed for another reason. A mass, swelling, or lump is sometimes felt around the area of a bone cancer. Bone fractures may also occur at the site of the bone tumor (including benign tumors as well as cancers) because the underlying bone has weakened. Other less common symptoms can result from compression or disruption of nerves and blood vessels in the affected area. These symptoms may include numbness, tingling, tenderness, or a decrease in blood flow (coldness, weakened pulse).

What are the different types of bone cancer?

There are several different types of bone cancer that can affect different patient populations, and they are often treated differently. Knowing the precise type of bone cancer is important to develop a customized management and treatment plan for the specific patient. Some of the most common types of bone cancer are discussed in the following section.

Osteosarcoma

Osteosarcoma is the most common type of bone cancer. Osteosarcoma occurs most commonly in teenagers and young adults (ages 10-19) and is more common in males. Osteosarcoma in young people tends to develop at the ends of long bones in areas of active bone growth, often around the knee, either at the end of the femur (thighbone) or the proximal part of the tibia (shinbone). The next most common location is in the bone of the upper arm, but it is possible for an osteosarcoma to develop in any bone. Depending on the appearance of the tumor cells under the microscope, there are several different subtypes of osteosarcoma.

Chondrosarcoma

Chondrosarcoma is the second most common bone cancer. It arises from cartilage cells. It is more common in people older than 40 years of age. It may be either rapidly growing and aggressive or slow-growing. Chondrosarcoma is most commonly found in the bones of the hips and pelvis.

Ewing sarcoma

Ewing sarcomas, sometimes referred to as the Ewing sarcoma family of tumors (ESFTs), are an aggressive form of bone cancer that is most common in children 4-15 years of age. It can occur either in the bones or in the soft tissues and is believed to arise from primitive nerve tissue. ESFTs are more common in males than in females and are extremely rare in African-American children. The most common location for Ewing sarcoma is the middle portion of the long bones of the arms and legs.

Pleomorphic sarcoma (malignant fibrous histiocytoma) of bone

Pleomorphic sarcoma is a tumor formerly referred to as malignant fibrous histiocytoma or MFH. This term is still used frequently. Pleomorphic sarcomas are typically tumors of the soft tissues, but in up to 5% of cases they may arise in the bone. Pleomorphic sarcomas typically occur in adults and can be found anywhere in the body.

Fibrosarcoma

Fibrosarcoma is an uncommon type of bone cancer. It is most commonly arises in adults behind the knee.

What kinds of bone cancer occur in children?

As mentioned above, osteosarcoma, the most common type of bone cancer, is most common in children and young adults. Ewing sarcoma is another bone cancer that typically affects children.

What tests are used to diagnose bone cancer?

A variety of imaging tests may be used to identify bone tumors, including bone cancers. Very early bone cancers may not be apparent on plain X-rays, but X-ray examination can identify many tumors. CT scans are more precise and may be used to identify bone tumors. MRI scans can provide detail of the soft tissues of the body and can typically reveal whether the cancer has spread beyond the bone into nearby tissues.

A bone scan is a test that uses radioactive material and X-rays to identify areas of rapidly growing or remodeling bone. This may allow the localization of tumors anywhere in the body. This test is not specific for bone cancers and can also reveal areas of inflammation as found with arthritis, fractures, and infections.

While many bone cancers have a characteristic appearance on imaging studies, a biopsy (tissue sample) must be taken in order to precisely determine what kind of cancer is present and confirm the diagnosis. The biopsy can be taken with a needle or through a surgical incision.

What is the treatment for bone cancer?

Surgical removal of the tumor is the mainstay of treatment for bone cancers. Improved surgical techniques allow for most bone cancers to be removed without requiring amputation of an affected limb. However, reconstructive surgery is often done in addition to tumor removal in order to maximize function.

For most types of bone cancers, chemotherapy drugs are given in addition to surgery. One exception is chondrosarcoma, which is typically not treated with chemotherapy. Radiation therapy is often used for chondrosarcomas and for Ewing sarcomas.

Ewing sarcomas that do not respond well to other treatments are sometimes treated with high-dose chemotherapy followed by a stem cell transplant. In this procedure, the patient's stem cells (blood cells that have the potential to make all the different kinds of blood cells) are harvested from the bloodstream in a way similar to a blood donation. After high doses of chemotherapy medications to destroy the bone marrow, the harvested stem cells are returned to the body as with a blood transfusion. Over the next three to four weeks, the stem cells produce new blood cells from the bone marrow.

How is bone cancer pain managed?

The pain of bone cancer is medically treated with analgesic (pain-relieving medications). These may be nonprescription or prescription medications. Mild-to-moderate pain is treated with medications such as acetaminophen (Tylenol) or nonsteroidal anti-inflammatory medications (NSAIDs), including ibuprofen and naproxen. However, people taking anticancer chemotherapy drugs may need to avoid the use of NSAIDs due to the increased risk of bleeding.

Prescription medications may be used for moderate-to-severe cancer pain. Opioids -- stronger narcotic pain medications -- like codeine, morphine, oxycodone (Oxycontin), hydromorphone (Dilaudid), and fentanyl may be required to control severe pain. Sometimes a combination of medications is used to treat cancer pain. Opioid medications can be associated with side effects like drowsiness, constipation, and nausea.

What is the prognosis for bone cancer?

The prognosis, or outlook, for bone cancer depends upon the particular type of cancer and the extent to which it has spread. The overall five-year survival rate for all bone cancers in adults and children is about 70%. Chondrosarcomas in adults have an overall five-year survival rate of about 80%.

The five-year survival rate specifically for localized osteosarcomas is about 60%-80%. If metastases are present, meaning that the cancer has spread beyond bone, the survival rate is about 15%-30%, but there are factors that may affect the prognosis. Osteosarcomas tend to have a more favorable prognosis if they are located in an arm or leg, respond well to chemotherapy, and are able to be completely removed at surgery. Younger patients and females also tend to have a more favorable prognosis than males or older patients.

Ewing sarcomas have a five-year survival rate of about 70% when they are found in a localized stage. If they have spread to other sites (metastasized), the survival rate drops to 15%-30%. Certain factors have been associated with a more favorable prognosis for Ewing sarcomas, including smaller tumor size, patient age less than 10, having a tumor in an arm or leg (in contrast to a tumor in the pelvis or chest wall), and having a good response to chemotherapy drugs.

Source: http://www.rxlist.com

As with other cancers, there is no one cause of bone cancer. In general, cancers arise when pathways that control normal cell growth and proliferation are disrupted, allowing abnormal cells to divide and grow uncontrollably. A number of hereditary and environmental factors are likely involved in the development of bone cancers.

Source: http://www.rxlist.com

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