SOG | Osteosarcoma

Osteosarcoma

What is Osteosarcoma (Bone Cancer)?

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.
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 asHereditary 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.
Stages of Osteosarcoma and Malignant Fibrous Histiocytoma of Bone

After osteosarcoma or malignant fibrous histiocytoma (MFH) has been diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.

The process used to find out if cancer has spread to other parts of the body is called staging. For osteosarcoma and malignant fibrous histiocytoma (MFH), most patients are grouped according to whether cancer is found in only one part of the body or has spread. The following tests and procedures may be used:

CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest, 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).
Chest x-ray: An x-ray of the organs and bones inside the chest. 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.
Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.
There are three ways that cancer spreads in the body. The three ways that cancer spreads in the body are:

Through tissue. Cancer invades the surrounding normal tissue.
Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.

When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if bone cancer spreads to the lung, the cancer cells in the lung are actually bone cancer cells. The disease is metastatic bone cancer, not lung cancer.
Osteosarcoma and MFH are described as either localized or metastatic.
Localized osteosarcoma or MFH has not spread out of the bone where the cancer started. There may be one or more areas of cancer in the bone that can be removed during surgery.
Metastatic osteosarcoma or MFH has spread from the bone in which the cancer began to other parts of the body. The cancer most often spreads to the lungs. It may also spread to other bones.

Recurrent Osteosarcoma and Malignant Fibrous Histiocytoma of Bone

Recurrent osteosarcoma and malignant fibrous histiocytoma (MFH) of bone are cancers that have recurred (come back) after being treated. The cancer may come back in the bone or in other parts of the body. Osteosarcoma and MFH most often recur in the lung, bone, or both. When osteosarcoma recurs, it is usually within 18 months after treatment is completed.

Treatment Option Overview

There are different types of treatment for patients with osteosarcoma or malignant fibrous histiocytoma (MFH) of bone.
Different types of treatment are available for children with osteosarcoma or malignant fibrous histiocytoma (MFH) of bone. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
Children with osteosarcoma or MFH should have their treatment planned by a team of health care providers with expertise in treating cancer in children.
Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric health care providers who are experts in treating osteosarcoma and MFH and who specialize in certain areas of medicine. These may include the following specialists:

Orthopedic surgeon
Radiation oncologist
Rehabilitation specialist
Pediatric nurse specialist
Social worker
Psychologist

Some cancer treatments cause side effects months or years after treatment has ended.
Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:

Physical problems.
Changes in mood, feelings, thinking, learning, or memory.
Second cancers (new types of cancer).

Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child.
Four types of standard treatment are used:

Surgery to remove the entire tumor will be done when possible. Chemotherapy may be given first, to make the tumor smaller so less tissue and bone needs to be removed. This is called neoadjuvant chemotherapy.

The following types of surgery may be done:

Wide local excision: Surgery to remove the cancer and some healthy tissue around it.
Limb-sparing surgery: Removal of the tumor in a limb (arm or leg) without amputation, so the use and appearance of the limb is saved. Most patients with osteosarcoma in a limb can be treated with limb-sparing surgery. The tumor is removed by wide local excision. Tissue and bone that are removed may be replaced with a graft using tissue and bone taken from another part of the patient's body, or with an implant such as artificial bone. If a fracture is found at diagnosis or during chemotherapy before surgery, limb-sparing surgery may still be possible in some cases. If the surgeon is not able to remove all of the tumor and enough healthy tissue around it, an amputation may be done.
Amputation: Surgery to remove part or all of an arm or leg. This may be done when it is not possible to remove all of the tumor in limb-sparing surgery. The patient may be fitted with a prosthesis (artificial limb) after amputation.
Rotationplasty: Surgery to remove the tumor and the knee joint. The part of the leg that remains below the knee is then attached to the part of the leg that remains above the knee, with the foot facing backward and the ankle acting as a knee. A prosthesis may then be attached to the foot.

Studies have shown that survival is the same whether the first surgery done is a limb-sparing surgery or an amputation.

Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery to lower the risk that the cancer will come back is called adjuvant therapy.

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is placed directly into the spinal column, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug. The way the chemotherapy is given depends on the type and stage of the cancer being treated.

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated.

Osteosarcoma and MFH cells are not killed easily by radiation therapy. It may be used when a small amount of cancer is left after surgery or used together with other treatments.

Samarium followed by stem cell transplant - Samarium is a radioactive drug that targets areas where bone cells are growing, such as tumor cells in bone. It helps relieve pain caused by cancer in the bone and it also kills blood cells in the bone marrow. Before treatment with samarium, stem cells (immature blood cells) are removed from the blood or bone marrow of the patient and are frozen and stored. After treatment with samarium is complete, the stored stem cells are thawed and given back to the patient through an infusion. These reinfused stem cells grow into (and restore) the body's blood cells.

New types of treatment are being tested in clinical trials.

Biologic therapy is a treatment that uses the patient’s immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body’s natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.

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