Genetic disorders

A very small number of bone cancers (especially osteosarcomas) appear to be hereditary.

Osteosarcoma

Children with certain rare inherited syndromes have an increased risk of developing osteosarcoma.

* The Li-Fraumeni syndrome makes people much more likely to develop several types of cancer, including breast cancer, brain cancer, osteosarcoma, and other types of sarcoma. Most of those cases are caused by a mutation of the p53 tumor suppressor gene, but some are caused by mutations in the gene CHEK2.

* Another syndrome that includes bone cancer is the Rothmund-Thompson syndrome. Children with this syndrome are short, have skeletal problems, and rashes. They also are more likely to develop osteosarcoma. This syndrome is caused by abnormal changes in the gene REQL4.

* Retinoblastoma is a rare eye cancer of 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.

Finally, there are families with several members that have developed osteosarcoma without inherited changes in any of the known genes. The gene defects that may cause cancers in these families haven’t been discovered yet.

Chondrosarcoma

Multiple exostoses (sometimes called multiple osteochondromas) is an inherited condition that causes many bumps on a person’s bones. These bumps are made mostly of cartilage. They can be painful and cause bones to deform and/or fracture. This disorder is caused by a mutation in any one of the 3 genes EXT1, EXT2, or EXT3. Patients with this condition have an increased risk of chondrosarcoma.

Paget disease

Paget disease is a benign (non-cancerous) but precancerous condition that affects one or more bones. It results in formation of abnormal bone tissue and is mostly a disease of people older than 50. Affected bones are heavy, thick, and brittle. They are weaker than normal bones and more likely to fracture (break). Most of the time Paget disease is not life threatening. Bone cancer (usually osteosarcoma) develops in about 1% of those with Paget disease, usually when many bones are affected.

Multiple enchondromatosis

An enchondroma is a benign cartilage tumor. People who get many of these tumors have a condition called multiple enchondromatosis. They have an increased risk of developing chondrosarcoma.

Radiation

Bone exposure to radiation may also increase the 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 at a younger age and/or being treated with higher doses of radiation (usually over 60 Gy) increases the risk of developing bone cancer. Exposure to radioactive materials such as radium and strontium can also cause bone cancer because these minerals build up in bones. Non-ionizing radiation, such as microwaves, electromagnetic fields from power lines, cellular phones, and household appliances, does not increase bone cancer risk.

Bone marrow transplantation

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

Injuries

People have wondered whether injury to a bone can cause cancer, but this has never been proven. Many people with bone cancer remember having hurt that part of their bone. Most doctors believe that this did not cause the cancer, but that the cancer caused them to remember the incident, or that the injury drew their attention to that bone and caused them to notice a problem that had already been present for some time.

Source: cancer.org

Osteosarcoma and chondrosarcoma are the 2 most common primary bone cancers (each one is about 35% of cases), These are followed by Ewing tumor (10%), chordoma (5%), and malignant fibrous histiocytoma/fibrosarcoma (2%). Several rare types of cancers account for the remainder of cases.

The prognosis (outlook) for people with primary bone cancer varies greatly, depending on the specific type of cancer and how far it has spread. If you have questions about your personal chances of cure of bone cancer or how long you might survive such a cancer, talk with your cancer care team.

Source: cancer.org

Bone is the supporting framework of the body. Most bones are hollow. The outer part of bones consists of a network of fibrous tissue called matrix onto which calcium salts are laid down. The soft tissue inside hollow bones is called bone marrow. At each end of the bone is a zone of, a softer form of bone-like tissue called cartilage.

Cartilage is made of a fibrous tissue matrix mixed with a gel-like substance that does not contain much calcium. Cartilage is softer than bone but more firm than most tissues.

Most bones start out as cartilage. The body then lays calcium down onto the cartilage to form bone. After the bone is formed, some cartilage may remain at the ends to act as a cushion between bones. This cartilage, along with ligaments and some other tissues connect bones to form a joint. In adults, cartilage is mainly found at the end of some bones as part of a joint. It is also seen at the place in the chest where the ribs meet the sternum (breastbone) and in parts of the face. The trachea (windpipe), larynx (voicebox), and the outer part of the ear are other structures that contain cartilage.

Bone itself is very hard and strong. Some bone is able to support as much as 12,000 pounds per square inch. It takes as much as 1,200 to 1,800 pounds of pressure to break the femur (thigh bone). The outside of the bone is covered with a layer of fibrous tissue called periosteum. The bone itself contains 2 kinds of cells. The osteoblast is the cell that lays down new bone, and the osteoclast is the cell that dissolves old bone. Although bone often looks like it doesn’t change much, the truth is that it is very active. Throughout our bodies, new bone is always forming while old bone is dissolving.

Inside hollow bones is a space called the medullary cavity where bone marrow is found. In some bones the marrow is only fatty tissue. The marrow in other bones is a mixture of fat cells and blood-forming cells. The blood-forming cells produce red blood cells, white blood cells, and blood platelets. Other cells in the marrow include plasma cells, fibroblasts, and reticuloendothelial cells.

Cells from any of these tissues can develop into a cancer.

Types of bone cancers

Most of the time when someone with cancer is told they have cancer in the bones, the doctor is talking about a cancer that spread there from somewhere else. This is called metastatic cancer. It can be seen in many different types of advanced cancer, such as breast cancer, prostate cancer, and lung cancer. When these cancers in the bone are looked at under a microscope, they resemble the tissue they came from. For example, if someone has lung cancer that has spread to bone, the cells of the cancer in the bone still look and act like lung cancer cells. They do not look or act like bone cancer cells, even though they are in the bones. Since these cancer cells still act like lung cancer cells, they still need to be treated with drugs that are used for lung cancer. For more information about metastatic bone cancer, please see the American Cancer Society document Bone Metastases, as well as the document on the specific place where the cancer started (Breast Cancer, Lung Cancer, Prostate Cancer, etc.).

Other kinds of cancers that are sometimes called “bone cancers” start in the blood forming cells of the bone marrow — not in the bone itself. The most common bone cancer is called multiple myeloma. Another cancer that starts in the bone marrow is leukemia, although it is generally considered a “blood cancer” rather than a “bone cancer.” Sometimes, lymphomas, which more often start in lymph nodes, can start in bone marrow. Multiple myeloma, lymphoma, and leukemia are not discussed in this document. For more information on these cancers, refer to the individual document for each.

A primary bone tumor is a tumor that starts in the bone itself. The main type of cancers that are true (or primary) “bone” cancers are called sarcomas. This is a term that describes the type of tissue that the cancer started in. Sarcomas are cancers that start in bone, muscle, fibrous tissue, blood vessels, fat tissue, as well as some other tissues. They can develop anywhere in the body.

There are several different types of bone tumors. Their names are based on the area of bone or surrounding tissue that is affected and the kind of cells forming the tumor. Some primary bone tumors are benign (not cancerous), and others are malignant (cancerous). Most bone cancers are called sarcomas.

Benign bone tumors

Benign tumors do not spread to other tissues and organs and so are not usually life threatening. They are generally cured by surgery. Types of benign bone tumors include:

* osteoid osteoma
* osteoblastoma
* osteochondroma
* enchondroma
* chondromyxoid fibroma.

These benign tumors are not discussed further in this document, which is limited to bone cancers.

Malignant bone tumors

Osteosarcoma: Osteosarcoma (also called osteogenic sarcoma) is the most common primary bone cancer. This cancer starts in the bone cells. It most often occurs in young people between the age of 10 and 30, but about 10% of osteosarcoma cases develop in people in their 60s and 70s. It is rare during middle age, and is more common in males than females. These tumors develop most often in bones of the arms, legs, or pelvis. For more information, see the American Cancer Society document, Osteosarcoma.

Chondrosarcoma: Chondrosarcoma is a cancer of cartilage cells. It is the second most common primary bone cancer. This cancer is rare in people younger than 20. After age 20, the risk of getting a chondrosarcoma goes up until about age 75. Women get this cancer as often as men.

Chondrosarcomas can develop in any place where there is cartilage. Most develop in bones such as the pelvis, leg bone or arm bone. Occasionally, chondrosarcoma will develop in the trachea, larynx, and chest wall. Other sites are the scapula (shoulder blade), ribs, or skull.

Benign (non-cancerous) tumors of cartilage are more common than malignant ones. These are called enchondromas. Another type of benign tumor that has cartilage is a bony projection capped by cartilage called an osteochondroma. These benign tumors rarely turn into cancer. There is a slightly higher chance of cancer developing in people who have many of these tumors, but this is still not common.

Chondrosarcomas are classified by grade, which measures how fast they grow. The grade is assigned by the pathologist (a doctor specially trained to examine and diagnose tissue samples under a microscope) after looking at the tumor under the microscope. The lower the grade, the slower the cancer grows. When a cancer is slow growing, the chance that it will spread is lower and so the outlook is better. Most chondrosarcomas are either low grade (grade I) or intermediate grade (grade II). High grade (grade III) chondrosarcomas, which are the most likely to spread, are less common.

Some chondrosarcomas have distinctive features under a microscope. These variants of chondrosarcoma can have a different prognosis (outlook) than usual chondrosarcomas.

* Dedifferentiated chondrosacromas start out as typical chondrosarcomas but then some parts of the tumor change into cells like those of an osteosarcoma or fibrosarcoma. This variant of chondrosarcoma tends to occur in older patients and is more aggressive than usual chondrosarcomas.

* Clear cell chondrosarcoma is a rare variant that grows slowly. It rarely spreads to other parts of the body unless it has already come back several times in the original location.

* Mesenchymal chondrosarcomas can grow rapidly, but like Ewing tumor, are sensitive to treatment with radiation and chemotherapy.

Ewing tumor: Ewing tumor is the third most common primary bone cancer. This cancer (also called Ewing sarcoma) is named after the doctor who first described it in 1921, Dr. James Ewing. 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. This cancer is most common in children and teenagers and is rare in adults over age 30. Ewing tumors occur most often in white people and are rare among African Americans and Asian Americans. More detailed information about this cancer can be found in the American Cancer Society document, Ewing Family of Tumors.

Malignant fibrous histiocytoma: Malignant fibrous histiocytoma (MFH) more often starts in “soft tissue” (connective tissue such as ligaments, tendons, fat, and muscle) than in bones. When MFH occurs in bones, it usually affects the legs (often around the knees) or arms. This cancer most often occurs in elderly and middle-aged adults and is rare among children. MFH tends to grow quickly and often spreads to other parts of the body, like the lungs and lymph nodes. MFH mostly tends to grow locally, but it can spread to distant sites

Fibrosarcoma: This is another type of cancer that develops more often in “soft tissues” than it does from bones. Fibrosarcoma usually occurs in elderly and middle-aged adults. Leg, arm, and jaw bones are the ones most often affected.

Giant cell tumor of bone: This type of primary bone tumor has benign and malignant forms. The benign (non-cancerous) form is most common. Giant cell bone tumors typically affect the leg (usually, near the knees) or arm bones of young and middle-aged adults. They don’t often spread to distant sites, but tend to come back where they started after surgery (this is called local recurrence). This can happen several times. With each recurrence, the tumor becomes more likely to spread to other parts of the body. Rarely, a giant cell bone tumor spreads to other parts of the body without first recurring locally. This happens in the malignant (cancer) form of the tumor.

Chordoma: This primary tumor of bone usually occurs in the base of the skull and bones of the spine. It develops most often in adults older than 30 years, and is about twice as common in men than in women. Chordomas tend to grow slowly and often do not spread to other parts of the body, but they often come back in the same area if they are not removed completely. When they do spread, lymph nodes, the lungs, and the liver are the most common areas for secondary tumors.

Other cancers that develop in bones

Non-Hodgkin lymphoma

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 multiple sites in the body are usually involved. The outlook is similar to other non-Hodgkin lymphomas of the same subtype and stage. Primary lymphoma of the bone is given the same treatment as lymphomas that start in lymph nodes — it is not treated like a primary bone sarcoma. For more information see the American Cancer Society document, Non-Hodgkin Lymphoma.

Multiple myeloma

Multiple myeloma is almost always found in bones, but doctors do not consider it a bone cancer because it develops from the plasma cells of the bone marrow (the soft inner part of some bones). Although it causes bone destruction, it is no more a bone cancer than is leukemia. It is treated as a widespread disease. At times, myeloma can be first found as a single tumor (called a plasmacytoma) in a single bone, but most of the time it will go on to spread to the marrow of other bones. For more information see the American Cancer Society document, Multiple Myeloma.

Source: cancer.org