Key Points for This Section

Osteosarcoma and malignant fibrous histiocytoma (MFH) of the bone are diseases in which malignant (cancer) cells form in bone. Being a male teenager or having past treatment with radiation can increase the risk of osteosarcoma. Possible signs of osteosarcoma and MFH include pain and swelling over a bone or a bony part of the body. Imaging tests are used to detect (find) osteosarcoma and MFH. A biopsy is done to diagnose osteosarcoma. Certain factors affect prognosis (chance of recovery) and treatment options.

Osteosarcoma and malignant fibrous histiocytoma (MFH) of the bone are diseases in which malignant (cancer) cells form in bone.

Osteosarcoma usually starts in osteoblasts, which are a type of bone cell that grows into new bone tissue. Osteosarcoma is most common in teenagers and young adults. It commonly forms in the ends of the long bones of the body, which include bones of the arms and legs. In children and teenagers, it often develops around the knee. Rarely, osteosarcoma may be found in soft tissue or organs in the chest or abdomen.

Osteosarcoma is the most common type of bone cancer. Malignant fibrous histiocytoma (MFH) of bone is a rare tumor of the bone. It is treated like osteosarcoma.

Ewing sarcoma is another kind of bone cancer, but it is not covered in this summary. See the PDQ summary on Ewing Family of Tumors Treatment ¹ for more information.

Being a male teenager or having past treatment with radiation can increase the risk of osteosarcoma.

Anything that increases your risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn’t mean that you will not get cancer. People who think they may be at risk should discuss this with their doctor. Risk factors for osteosarcoma include the following:

• Being a teen or young adult. Osteosarcoma and MFH often form during a growth spurt.
• Being male.
• Past treatment with radiation therapy.
• Past treatment with anticancer drugs called alkylating agents.
• Having a certain change in the retinoblastoma gene.
• Having certain conditions such as
  • Hereditary retinoblastoma.
  • Li-Fraumeni syndrome.
  • Rothmund-Thomson syndrome.
  • Paget disease.

Possible signs of osteosarcoma and MFH include pain and swelling over a bone or a bony part of the body.

These and other symptoms may be caused by osteosarcoma or MFH. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur:

• Swelling over a bone or bony part of the body.
• Pain in a bone or joint.
• A bone that breaks for no known reason.

Imaging tests are used to detect (find) osteosarcoma and MFH.

Imaging tests are done before the biopsy. The following tests and procedures may be used:

• Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
• X-ray: An x-ray of the organs and bones inside the body. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
• CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
• MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI).

A biopsy is done to diagnose osteosarcoma.

Cells and tissues are removed during a biopsy so they can be viewed under a microscope by a pathologist to check for signs of cancer. It is important that the biopsy be done by a surgeon who is an expert in treating cancer of the bone. It is best if that surgeon is also the one who removes the tumor. The biopsy and the surgery to remove the tumor are planned together. The way the biopsy is done affects which type of surgery can be done later.

The type of biopsy that is done will be based on the size of the tumor and where it is in the body. There are three types of biopsy that may be used:

• Fine-needle aspiration (FNA) biopsy: The removal of tissue or fluid using a thin needle.
• Core biopsy: The removal of tissue using a wide needle.
• Incisional biopsy: The removal of part of a lump or a sample of tissue that doesn't look normal.

The following tests may be done on the tissue that is removed:

• Light and electron microscopy: A laboratory test in which cells in a sample of tissue are viewed under regular and high-powered microscopes to look for certain changes in the cells.
• Cytogenetic analysis: A laboratory test in which cells in a sample of tissue are viewed under a microscope to look for certain changes in the chromosomes.
• Immunocytochemistry study: A laboratory test in which a substance such as an antibody, dye, or radioisotope is added to a sample of cancer cells to test for certain antigens. This type of study is used to tell the difference between different types of cancer.

Certain factors affect prognosis (chance of recovery) and treatment options.

The prognosis (chance of recovery) is affected by certain factors before and after treatment.

The prognosis of untreated osteosarcoma and MFH depends on the following:

• Where the tumor is in the body and whether tumors formed in more than one bone.
• The size of the tumor.
• Whether the cancer has spread to other parts of the body and where it has spread.
• The age of the patient.
• The type of tumor (based on how the cancer cells look under a microscope).
• Whether the patient has certain genetic diseases.

After osteosarcoma or MFH is treated, prognosis also depends on the following:

• How much of the cancer was killed by chemotherapy.
• How much of the tumor was taken out by surgery.

Treatment options for osteosarcoma and MFH depend on the following:

• Where the tumor is in the body.
• The size of the tumor.
• The stage of the cancer.
• Whether the bones are still growing.
• The patient's age and general health.
• The desire of the patient and family for the patient to be able to participate in activities such as sports or have a certain appearance.
• Whether the cancer is newly diagnosed or has recurred (come back) after treatment.

Glossary Terms

The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.

A type of drug that is used in the treatment of cancer. It interferes with the cell's DNA and inhibits cancer cell growth.

A protein made by plasma cells (a type of white blood cell) in response to an antigen (a substance that causes the body to make a specific immune response). Each antibody can bind to only one specific antigen. The purpose of this binding is to help destroy the antigen. Some antibodies destroy antigens directly. Others make it easier for white blood cells to destroy the antigen.

Any substance that causes the body to make a specific immune response.

The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.

Primary bone cancer is cancer that forms in cells of the bone. Some types of primary bone cancer are osteosarcoma, Ewing sarcoma, malignant fibrous histiocytoma, and chondrosarcoma. Secondary bone cancer is cancer that spreads to the bone from another part of the body (such as the prostate, breast, or lung).

A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord. Also called malignancy.

The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.

Treatment with drugs that kill cancer cells.

Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.

The removal of a tissue sample with a wide needle for examination under a microscope. Also called core needle biopsy.

A series of detailed pictures of areas inside the body taken from different angles. The pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.

The study of chromosomes and chromosomal abnormalities.

Any substance, other than food, that is used to prevent, diagnose, treat or relieve symptoms of a disease or abnormal condition. Also refers to a substance that alters mood or body function, or that can be habit-forming or addictive, especially a narcotic.

A microscope (device used to magnify small objects) that uses electrons (instead of light) to produce an enlarged image. An electron microscope shows tiny details better than any other type of microscope.

A type of cancer that forms in bone or soft tissue. Also called peripheral primitive neuroectodermal tumor and pPNET.

The removal of tissue or fluid with a thin needle for examination under a microscope. Also called FNA biopsy.

A substance that flows smoothly and takes the shape of its container. Liquids and gases are fluids.

The functional and physical unit of heredity passed from parent to offspring. Genes are pieces of DNA, and most genes contain the information for making a specific protein.

Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.

Transmitted from parent to child by information contained in the genes.

In medicine, a process that makes pictures of areas inside the body. Imaging uses methods such as x-rays (high-energy radiation), ultrasound (high-energy sound waves), and radio waves.

A surgical procedure in which a portion of a lump or suspicious area is removed for diagnosis. The tissue is then examined under a microscope to check for signs of disease.

Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."

In medicine, the place where two or more bones are connected. Examples include the shoulder, elbow, knee, and jaw.

A medical procedure that involves testing a sample of blood, urine, or other substance from the body. Tests can help determine a diagnosis, plan treatment, check to see if treatment is working, or monitor the disease over time.

A rare, inherited predisposition to multiple cancers, caused by an alteration in the p53 tumor suppressor gene.

A microscope (device to magnify small objects) in which objects are lit directly by white light.

A soft tissue sarcoma that usually occurs in the limbs, most commonly the legs, and may also occur in the abdomen. Also called malignant fibrous cytoma.

An instrument that is used to look at cells and other small objects that cannot be seen with the eye alone.

A procedure in which radio waves and a powerful magnet linked to a computer is used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, NMRI, and nuclear magnetic resonance imaging.

A part of the body that performs a specific function. For example, the heart is an organ.

A cancer of the bone that usually affects the large bones of the arm or leg. It occurs most commonly in young people and affects more males than females. Also called osteogenic sarcoma.

A doctor who identifies diseases by studying cells and tissues under a microscope.

PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.

An exam of the body to check for general signs of disease.

The likely outcome or course of a disease; the chance of recovery or recurrence.

The use of high-energy radiation from x-rays, gamma rays, neutrons, protons, and other sources to kill cancer cells and shrink tumors. Radiation may come from a machine outside the body (external-beam radiation therapy), or it may come from radioactive material placed in the body near cancer cells (internal radiation therapy). Systemic radiation therapy uses a radioactive substance, such as a radiolabeled monoclonal antibody, that travels in the blood to tissues throughout the body. Also called irradiation and radiotherapy.

An unstable form of a chemical element that releases radiation as it breaks down and becomes more stable. Radioisotopes may occur in nature or be made in a laboratory. In medicine, they are used in imaging tests and in treatment. Also called radionuclide.

To come back or to return.

Cancer that forms in the tissues of the retina (the light-sensitive layers of nerve tissue at the back of the eye). Retinoblastoma usually occurs in children younger than 5 years. It may be hereditary or nonhereditary (sporadic).

Something that increases the chance of developing a disease. Some examples of risk factors for cancer are age, a family history of certain cancers, use of tobacco products, being exposed to radiation or certain chemicals, infection with certain viruses or bacteria, and certain genetic changes.

Refers to muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.

The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.

A doctor who removes or repairs a part of the body by operating on the patient.

A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.

An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.

A set of symptoms or conditions that occur together and suggest the presence of a certain disease or an increased chance of developing the disease.

A group or layer of cells that work together to perform a specific function.

An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.

A blood vessel that carries blood to the heart from tissues and organs in the body.

A type of high-energy radiation. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer.