21st Century Pediatric Cancer Sourcebook: Childhood Bone Cancer - Osteosarcoma and Malignant Fibrous Histiocytoma (MFH) of Bone - Clinical Data, Practical Information for Patients, Physicians
Edition 1.0 - March 2011
National Cancer Institute
Smashwords Edition
Copyright 2011 Progressive Management
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OncoLink * http://www.oncolink.upenn.edu/
eMedicine.com * http://www.emedicine.com/
American Cancer Society (ACS) * http://www.cancer.org/
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PART ONE
Chapter 1A: Osteosarcoma and Malignant Fibrous Histiocytoma of Bone
PART TWO
Chapter 1B Late Effects of Treatment for Childhood Cancer - Patient Version
Chapter 2B: Pediatric Supportive Care
Chapter 3B: Clinical Trials Background Information
Chapter 4B: Cancer Clinical Trials -The Basic Workbook
Chapter 5B: Cancer Clinical Trials - The In-Depth Program
Chapter 6B: Clinical Trials at NIH
Chapter 7B: How To Find A Cancer Treatment Trial: A Ten Step Guide
Chapter 8B: Taking Part in Cancer Treatment Research Studies
Chapter 9B: Cancer Clinical Trials
Chapter 10B: Access to Investigational Drugs
Chapter 12B: Taking Time: Support for People with Cancer
Chapter 13B: Facing Forward - Life After Cancer Treatment
Chapter 14B: When Someone You Love Is Being Treated For Cancer
Chapter 15B: Living Beyond Cancer: Finding a New Balance
Chapter 16B: Caring for the Caregiver
Chapter 17B: Young People With Cancer, A Handbook For Parents
Chapter 18B: When Cancer Returns
Chapter 19B: When Someone You Love Has Advanced Cancer / Support for Caregivers
Chapter 20B: Chemotherapy and You
Chapter 21B: Managing Chemotherapy Side Effects - Anemia
Chapter 22B: Managing Chemotherapy Side Effects - Appetite Changes
Chapter 23B: Managing Chemotherapy Side Effects - Bleeding Problems
Chapter 24B: Managing Chemotherapy Side Effects - Constipation
Chapter 25B: Managing Chemotherapy Side Effects - Memory Changes
Chapter 26B: Managing Chemotherapy Side Effects - Mouth and Throat Changes
Chapter 27B: Managing Chemotherapy Side Effects - Nerve Changes
Chapter 28B: Managing Chemotherapy Side Effects - Pain
Chapter 29B: Managing Chemotherapy Side Effects - Skin and Nail Changes
Chapter 30B: Managing Chemotherapy Side Effects - Swelling (Fluid retention)
Chapter 31B: Targeted Cancer Therapies
Chapter 33B : Follow-up Care After Cancer Treatment
Chapter 34B: Radiation Therapy and You
Chapter 36B: Understanding Radiation Therapy, What To Know About External Beam Radiation Therapy
Chapter 37B: Radiation Therapy for Cancer
Chapter 38B: Managing Radiation Therapy Side Effects - What To Do When Your Mouth or Throat Hurts
Chapter 39B: What To Do About Hair Loss (Alopecia)
Chapter 42B: Managing Radiation Therapy Side Effects - Changes When You Urinate
Chapter 43B: Managing Radiation Therapy Side Effects What To Do About Mild Skin Changes
Chapter 45B: General Cancer Information And Resources
Chapter 46B: Cancer And The Environment - What You Need to Know, What You Can Do
Chapter 48B: FDA Warning: Beware of Online Cancer Fraud
Chapter 49B: FDA Office of Oncology Drug Products
Chapter 50B: Understanding the HIPAA Privacy Rule
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Chapter 1A: Osteosarcoma and Malignant Fibrous Histiocytoma of Bone Treatment
Last Modified: 07/28/2010
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.
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. * Paget disease. * Diamond-Blackfan anemia. * Li-Fraumeni syndrome. * Rothmund-Thomson syndrome. * Bloom syndrome. * Werner syndrome.
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.
Whether chemotherapy is delayed for more than 3 weeks after surgery takes place.
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.
There are three ways that cancer spreads in the body.
Osteosarcoma and MFH are described as either localized or metastatic.
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:
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. X-rays will be taken of the chest and the area where the tumor formed.
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. Pictures will be taken of the chest and the area where the tumor formed.
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).
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.
PET scan (positron emission tomography scan): A procedure to find malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
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
Key Points for This Section
There are different types of treatment for patients with osteosarcoma or malignant fibrous histiocytoma (MFH) of bone.
Children with osteosarcoma or MFH should have their treatment planned by a team of health care providers with expertise in treating cancer in children.
Some cancer treatments cause side effects months or years after treatment has ended.
Four types of standard treatment are used:
Surgery * Chemotherapy * Radiation therapy * Samarium
New types of treatment are being tested in clinical trials.
Biologic therapy
Patients may want to think about taking part in a clinical trial.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Follow-up tests may be needed.
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
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
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 cerebrospinal fluid, 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
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
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.
Treatment with samarium may be followed by stem cell transplant. 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.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about ongoing clinical trials is available from the NCI Web site.
Biologic therapy
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.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Osteosarcoma and Malignant Fibrous Histiocytoma of Bone
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.
Localized Osteosarcoma and Malignant Fibrous Histiocytoma of Bone
Treatment may include the following:
Surgery followed by adjuvant chemotherapy or radiation therapy. Most patients also receive chemotherapy before the surgery.
Radiation therapy when the tumor cannot be completely removed by surgery.
A clinical trial of chemotherapy given before and after surgery, with or without biologic therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with localized osteosarcoma and localized childhood malignant fibrous histiocytoma of bone. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Metastatic Osteosarcoma and Malignant Fibrous Histiocytoma of Bone
Lung Metastasis
When osteosarcoma or malignant fibrous histiocytoma (MFH) spread, it usually spreads to the lung. Treatment of osteosarcoma and MFH with lung metastasis is usually chemotherapy followed by surgery to remove the cancer that has spread to the lung.
Bone Metastasis or Bone with Lung Metastasis
Osteosarcoma and malignant fibrous histiocytoma may spread to bone and/or the lung. Treatment may include the following:
Chemotherapy followed by surgery to remove the cancer followed by combination chemotherapy.
Surgery to remove the primary tumor followed by chemotherapy and surgery to remove cancer that has spread to other parts of the body.
A clinical trial of chemotherapy given before and after surgery, with or without biologic therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with metastatic osteosarcoma and metastatic childhood malignant fibrous histiocytoma of bone. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Recurrent Osteosarcoma and Malignant Fibrous Histiocytoma of Bone
Treatment of recurrent osteosarcoma and malignant fibrous histiocytoma of bone may include the following:
Surgery to remove the tumor with or without chemotherapy.
Samarium with or without stem cell transplant using the patient's own stem cells, as palliative treatment to relieve pain and improve the quality of life.
A clinical trial of new types of treatment for patients whose cancer cannot be removed by surgery.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent osteosarcoma 9 and recurrent childhood malignant fibrous histiocytoma of bone 10. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site.
Glossary Terms
abdomen (AB-doh-men)
The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.
alkylating agent (AL-kuh-LAY-ting AY-jent)
A type of drug that is used in the treatment of cancer. It interferes with the cell's DNA and inhibits cancer cell growth.
antibody (AN-tee-BAH-dee)
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.
antigen (AN-tih-jen)
Any substance that causes the body to make a specific immune response.
biopsy (BY-op-see)
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.
Bloom syndrome (… SIN-drome)
A rare, inherited disorder marked by height that is shorter than average, a narrow face with redness and a rash, a high-pitched voice, and fertility problems. Patients with this disorder have an increased risk of cancer, especially leukemia and osteosarcoma (bone cancer). Bloom syndrome is caused by changes in a protein that normally helps cells make copies of the DNA. Changes in this protein cause many breaks, rearrangements, and other mutations in the DNA. It is a type of autosomal recessive genetic disease. Also called Bloom-Torre-Machacek syndrome.
bone cancer (bone KAN-ser)
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).
cancer (KAN-ser)
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.
cell (sel)
The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.
chemotherapy (KEE-moh-THAYR-uh-pee)
Treatment with drugs that kill cancer cells.
chromosome (KROH-muh-some)
Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.
core biopsy (... BY-op-see)
The removal of a tissue sample with a wide needle for examination under a microscope. Also called core needle biopsy.
CT scan
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.
cytogenetics (SY-toh-jeh-NEH-tix)
The study of chromosomes and chromosomal abnormalities.
Diamond-Blackfan anemia (DY-uh-mund-BLAK-fan uh-NEE-mee-uh)
A very rare disorder in which the bone marrow doesn’t make enough red blood cells. It is usually seen in the first year of life. Patients may have deformed thumbs and other physical problems. They also have an increased risk of leukemia and sarcoma, especially osteosarcoma (bone cancer). Patients with Diamond-Blackfan anemia may have a mutation (change) in one of the genes that make proteins found in the cell’s ribosomes. Also called Blackfan–Diamond anemia, congenital hypoplastic anemia, congenital pure red cell aplasia, DBA, erythrogenesis imperfecta, and inherited erythroblastopenia.
drug
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.
electron microscope (ee-LEK-tron MY-kroh-SKOPE)
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.
Ewing sarcoma (YOO-ing sar-KOH-muh)
A type of cancer that forms in bone or soft tissue. Also called peripheral primitive neuroectodermal tumor and pPNET.
fine-needle aspiration biopsy (... NEE-dul AS-pih-RAY-shun BY-op-see)
The removal of tissue or fluid with a thin needle for examination under a microscope. Also called FNA biopsy.
fluid (FLOO-id)
A substance that flows smoothly and takes the shape of its container. Liquids and gases are fluids.
gene (JEEN)
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.
genetic (jeh-NEH-tik)
Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.
hereditary (huh-REH-dih-tayr-ee)
Transmitted from parent to child by information contained in the genes.
imaging (IH-muh-jing)
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.
incisional biopsy (in-SIH-zhun-al BY-op-see)
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.
injection
Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."
joint (joynt)
In medicine, the place where two or more bones are connected. Examples include the shoulder, elbow, knee, and jaw.
laboratory test (LA-bruh-tor-ee...)
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.
Li-Fraumeni syndrome
A rare, inherited predisposition to multiple cancers, caused by an alteration in the p53 tumor suppressor gene.
light microscope (lite MY-kroh-SKOPE)
A microscope (device to magnify small objects) in which objects are lit directly by white light.
malignant fibrous histiocytoma (muh-LIG-nunt FY-brus HIS-tee-oh-sy-TOH-muh)
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.
microscope (MY-kroh-SKOPE)
An instrument that is used to look at cells and other small objects that cannot be seen with the eye alone.
MRI
A procedure in which radio waves and a powerful magnet linked to a computer are 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.
organ
A part of the body that performs a specific function. For example, the heart is an organ.
osteosarcoma (OS-tee-oh-sar-KOH-muh)
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.
Paget disease of bone (PA-jet dih-ZEEZ ...)
A chronic condition in which both the breakdown and regrowth of bone are increased. Paget disease of bone occurs most frequently in the pelvic and leg bones, skull, and lower spine. It is most common in older individuals, and may lead to bone pain, deformities, and fractures. Also called osteitis deformans.
pathologist (puh-THAH-loh-jist)
A doctor who identifies diseases by studying cells and tissues under a microscope.
physical examination (FIH-zih-kul eg-ZA-mih-NAY-shun)
An exam of the body to check for general signs of disease.
prognosis (prog-NO-sis)
The likely outcome or course of a disease; the chance of recovery or recurrence.
radiation therapy (RAY-dee-AY-shun THAYR-uh-pee)
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.
radioisotope (RAY-dee-oh-I-suh-tope)
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.
recur
To come back or to return.
retinoblastoma (REH-tih-noh-blas-TOH-muh)
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).
risk factor (... FAK-ter)
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.
Rothmund-Thomson syndrome (ROT-moond-TOM-sun SIN-drome)
A rare inherited disorder that affects the skin and many other parts of the body, including the bones, eyes, nose, hair, nails, teeth, testes, and ovaries. People with Rothmund-Thomson syndrome have an increased risk of osteosarcoma (bone cancer). Also called RTS.
soft tissue (... TIH-shoo)
Refers to muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.
stage
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.
surgeon (SER-jun)
A doctor who removes or repairs a part of the body by operating on the patient.
surgery (SER-juh-ree)
A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.
symptom
An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.
tissue (TISH-oo)
A group or layer of cells that work together to perform a specific function.
tumor (TOO-mer)
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.
vein (vayn)
A blood vessel that carries blood to the heart from tissues and organs in the body.
Werner syndrome (VER-nur SIN-drome)
An inherited disorder marked by rapid aging that begins in early adolescence. Patients may be shorter than average, and have health problems such as loss and graying of hair, hardening of the arteries, thinning of the bones, diabetes, and thin, hardened skin. They also have an increased risk of cancer, especially osteosarcoma (a type of bone cancer). Werner syndrome is caused by a mutation (change) in a gene involved in cell division. It is a type of autosomal recessive gene disease. Also called adult progeria and WS.
x-ray
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.
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Osteosarcoma/Malignant Fibrous Histiocytoma of Bone Treatment - Last Modified: 06/30/2010 - Health Professional Version
Prognostic Factors Primary Site * Tumor Size * Presence of Clinically Detectable Metastatic Disease * Adequacy of Tumor Resection * Necrosis Following Induction or Neoadjuvant Chemotherapy * Additional Prognostic Factors * Syndromes Associated with Osteosarcoma Rothmund-Thomson Syndrome (RTS) * Inherited Diseases That Predispose to Osteosarcoma * Cellular Classification Central (Medullary) Tumors * Surface (Peripheral) Tumors * Staging and Site Information Localized Osteosarcoma * Metastatic Osteosarcoma * Treatment Option Overview * Localized Osteosarcoma/Malignant Fibrous Histiocytoma of Bone Biopsy * Surgical Removal of Primary Tumor * Chemotherapy * Osteosarcoma of the Head and Neck * Treatment Options Under Clinical Evaluation * Current Clinical Trials * Osteosarcoma/Malignant Fibrous Histiocytoma of Bone with Metasta Lung Metastases Only * Bone Only or Bone with Lung Metastasis * Treatment Options Under Clinical Evaluation * Malignant Fibrous Histiocytoma of Bone * Current Clinical Trials * Recurrent Osteosarcoma/Malignant Fibrous Histiocytoma of Bone Lung Only Recurrence * Bone Metastases Only * Local Recurrence * Second Recurrence of Osteosarcoma * Treatment Options Under Clinical Evaluation for Recurrent Osteosarcoma * Current Clinical Trials
Fortunately, cancer in children and adolescents is rare, although the overall incidence of childhood cancer has been slowly increasing since 1975.[1]. Children and adolescents with cancer should be referred to medical centers that have a multidisciplinary team of cancer specialists with experience treating the cancers that occur during childhood and adolescence. This multidisciplinary team approach incorporates the skills of the primary care physician, an orthopedic surgeon experienced in bone tumors, a pathologist, radiation oncologists, pediatric oncologists, rehabilitation specialists, pediatric nurse specialists, social workers, and others to ensure that children receive treatment, supportive care, and rehabilitation that will achieve optimal survival and quality of life.
Guidelines for pediatric cancer centers and their role in the treatment of pediatric patients with cancer have been outlined by the American Academy of Pediatrics.[2] At these pediatric cancer centers, clinical trials are available for most types of cancer that occur in children and adolescents, and the opportunity to participate in these trials is offered to most patients/families. Clinical trials for children and adolescents with cancer are generally designed to compare potentially better therapy with therapy that is currently accepted as standard. Most of the progress made in identifying curative therapies for childhood cancers has been achieved through clinical trials. Information about ongoing clinical trials is available from the NCI Web site.
Dramatic improvements in survival have been achieved for children and adolescents with cancer. Between 1975 and 2002, childhood cancer mortality has decreased by more than 50%. For osteosarcoma, the 5-year survival rate has increased over the same time from 40% to 67% in children and adolescents.[1] Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment.
Osteosarcoma occurs predominantly in adolescents and young adults. Review of data from the Surveillance, Epidemiology and End Results program of the NCI resulted in an estimate of 4.4 per million new cases of osteosarcoma each year in people aged 0 to 24 years.[3] The U.S. Census Bureau estimates that there will be 110 million people in this age range in 2010, resulting in an incidence of roughly 450 cases per year in children and young adults less than 25 years old. Osteosarcoma accounts for approximately 5% of childhood tumors. In children and adolescents, more than 50% of these tumors arise from the bones around the knee. Osteosarcoma can rarely be observed in soft tissue or visceral organs. There appears to be no difference in presenting symptoms, tumor location, and outcome for younger patients (<10 years) compared with adolescents.[4,5] Two trials conducted in the 1980s were designed to address the natural history of surgically treated localized, resectable osteosarcoma of the extremity. The outcome of patients in these trials who were treated with surgical removal of the primary tumor recapitulated the historical experience before 1970; more than half of these patients developed metastases within 6 months of diagnosis, and overall, approximately 90% developed recurrent disease within 2 years of diagnosis.[6] Overall survival for patients treated with surgery alone was statistically inferior.[7] The natural history of osteosarcoma has not changed over time, and fewer than 20% of patients with localized resectable primary tumors treated with surgery alone can be expected to survive free of relapse.[6,8,9]
References
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Guidelines for the pediatric cancer center and role of such centers in diagnosis and treatment. American Academy of Pediatrics Section Statement Section on Hematology/Oncology. Pediatrics 99 (1): 139-41, 1997. [PUBMED Abstract]
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Prognostic Factors
Pretreatment factors that influence outcome include site and size of the primary tumor and presence or absence of clinically detectable metastatic disease.[1] After administration of preoperative chemotherapy, surgical resectability and the degree of tumor necrosis influence outcome. In general, prognostic factors in osteosarcoma have not been helpful in identifying patients who might benefit from treatment intensification or who might require less therapy while maintaining an excellent outcome.
Primary Site
The site of the primary tumor is a significant prognostic factor for patients with localized disease. Among extremity tumors, distal sites have a more favorable prognosis than proximal sites. Axial skeleton primary tumors are associated with the greatest risk of progression and death, primarily related to the inability to achieve a complete surgical resection. Pelvic osteosarcomas make up 7% to 9% of all osteosarcomas; survival rates for patients with pelvic primary tumors are 20% to 47%.[2,3] Complete surgical resection is associated with positive outcome for osteosarcoma of the pelvis.[4] Within the craniofacial osteosarcoma group, patients with mandibular tumors have a significantly better prognosis than patients with extragnathic tumors.[4] For patients with osteosarcoma of craniofacial bones, complete resection of the primary tumor with negative margins is essential for cure.[5-7] Despite a relatively high rate of inferior necrosis following neoadjuvant chemotherapy, fewer patients with craniofacial primaries develop systemic metastases than do patients with osteosarcoma originating in the extremities.[8-10] This low rate of metastasis may be related to the relatively smaller size and higher incidence of lower grade tumors in osteosarcoma of the head and neck. There is a better prognosis for patients who have osteosarcoma of the head and neck than for those who have appendicular lesions when treated with surgery alone. While small series have not shown a benefit from adjuvant chemotherapy for patients with osteosarcoma of the head and neck, one meta-analysis concluded that systemic chemotherapy improves the prognosis for these patients. Another large meta-analysis detected no benefit from chemotherapy for patients with osteosarcoma of the head and neck, but suggested that the incorporation of chemotherapy into treatment of patients with high-grade tumors may improve survival. A retrospective analysis identified a trend toward better survival in patients with high-grade osteosarcoma of the mandible and maxilla who received adjuvant chemotherapy.[7,11] Radiation therapy was found to improve local control, disease-specific survival, and overall survival (OS) in a retrospective study of osteosarcoma of the craniofacial bones, which had positive or uncertain resection margins after surgical resection.[12][Level of evidence: 3iiA] Radiation-associated craniofacial osteosarcomas are generally high-grade lesions, usually fibroblastic, that tend to recur locally with a high rate of metastasis.[13] In the German series, approximately 25% of patients with craniofacial osteosarcoma had osteosarcoma as a second tumor and 11 patients were postretinoblastoma. In this series, there was no difference in outcome for primary or secondary craniofacial osteosarcoma.[4]
Osteosarcoma in extraskeletal sites is rare in children and young adults. With current combined-modality therapy, the outcome for patients with extraskeletal osteosarcoma appears to be similar to that for patients with primary tumors of bone.[14]
Tumor Size
Larger tumors have a worse prognosis than smaller tumors. Tumor size has been assessed by the longest single dimension, by the cross-sectional area, or by an estimate of tumor volume; all have correlated with outcome. Serum lactate dehydrogenase (LDH), which also correlates with outcome, is a likely surrogate for tumor volume.
Presence of Clinically Detectable Metastatic Disease
Patients with localized disease have a much better prognosis than those with overt metastatic disease. As many as 20% of patients will have radiographically detectable metastases at diagnosis, with the lung being the most common site.[15] The prognosis for patients with metastatic disease appears to be determined largely by the site(s), the number of metastases, and the surgical resectability of the metastatic disease.[16,17] Patients who have complete surgical ablation of the primary and metastatic tumor (when confined to the lung) following chemotherapy may attain long-term survival, though overall event-free survival remains about 20% to 30% for patients with metastatic disease at diagnosis.[16-19] Prognosis appears more favorable for patients with fewer pulmonary nodules and for those with unilateral rather than bilateral pulmonary metastases;[16] not all patients with suspected pulmonary metastases at diagnosis have osteosarcoma confirmed at the time of lung resection. In one large series, approximately 25% of patients had exclusively benign lesions removed at the time of surgery.[17] The degree of necrosis in the primary tumor after induction chemotherapy remains prognostic in metastatic osteosarcoma.[20] Patients with skip metastases (at least two discontinuous lesions in the same bone) have been reported to have inferior prognoses.[21] Analysis of the German Cooperative Osteosarcoma Study experience, however, suggests that skip lesions in the same bone do not confer an inferior prognosis if they are included in planned surgical resection. Skip lesions across a joint have a worse prognosis.[22] Patients with multifocal osteosarcoma (defined as multiple bone lesions without a clear primary tumor) have an extremely poor prognosis.[23]
Adequacy of Tumor Resection
Resectability of the tumor is a critical prognostic feature because osteosarcoma is relatively resistant to radiation therapy. Complete resection of the primary tumor and any skip lesions with adequate margins is generally considered essential for cure. For patients with axial skeletal primaries who either do not have surgery for their primary tumor or who have surgery resulting in positive margins, radiation therapy may improve survival.[2,24]
Necrosis Following Induction or Neoadjuvant Chemotherapy
Most treatment protocols for osteosarcoma use an initial period of systemic chemotherapy prior to definitive resection of the primary tumor (or resection of sites of metastases for patients with metastatic disease). The pathologist assesses necrosis in the resected tumor. Patients with at least 90% [25] necrosis in the primary tumor after induction chemotherapy have a better prognosis than those with less necrosis.[25] Patients with less necrosis (<90%) in the primary tumor following initial chemotherapy have a higher rate of recurrence within the first 2 years compared with patients with a more favorable amount of necrosis (≥90%).[26] Imaging modalities such as dynamic magnetic resonance imaging (MRI) or positron emission tomography (PET) scanning are under investigation as noninvasive methods to assess response.[27-31] Less necrosis should not be interpreted to mean that chemotherapy has been ineffective; cure rates for patients with little or no necrosis following induction chemotherapy are much higher than cure rates for patients who receive no chemotherapy.
Additional Prognostic Factors
Patients with osteosarcoma as a second malignant neoplasm including those tumors arising in a radiation field, share the same prognosis as patients with de novo osteosarcoma if they are treated aggressively with complete surgical resection and multiagent chemotherapy.[32-35] There have been numerous other identified prognostic features for patients with conventional localized high-grade osteosarcoma. These factors include the age of the patient, LDH level, alkaline phosphatase level, and histologic subtype.[25,36-39] A number of potential prognostic factors have been identified but have not been tested in large numbers of patients. These include the expression of HER2/c-erbB-2 (there are conflicting data concerning the prognostic significance of this human epidermal growth factor);[40-42] tumor cell ploidy; specific chromosomal gains or losses;[43] loss of heterozygosity (LOH) of the RB gene;[44,45] LOH of the p53 locus;[46] and increased expression of p-glycoprotein.[47,48] A prospective analysis of p-glycoprotein expression determined by immunohistochemistry failed to identify prognostic significance for newly diagnosed patients with osteosarcoma, although earlier studies suggested that overexpression of p-glycoprotein predicted for poor outcome.[49] In a large series, a delay of 21 days or more from the time of definitive surgery to the resumption of chemotherapy was an adverse prognostic factor.[50] Pathologic fracture at diagnosis or during preoperative chemotherapy does not have adverse prognostic significance.[51]
References
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Ozaki T, Flege S, Kevric M, et al.: Osteosarcoma of the pelvis: experience of the Cooperative Osteosarcoma Study Group. J Clin Oncol 21 (2): 334-41, 2003. [PUBMED Abstract]
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Jasnau S, Meyer U, Potratz J, et al.: Craniofacial osteosarcoma Experience of the cooperative German-Austrian-Swiss osteosarcoma study group. Oral Oncol 44 (3): 286-94, 2008. [PUBMED Abstract]
Patel SG, Meyers P, Huvos AG, et al.: Improved outcomes in patients with osteogenic sarcoma of the head and neck. Cancer 95 (7): 1495-503, 2002. [PUBMED Abstract]
Smith RB, Apostolakis LW, Karnell LH, et al.: National Cancer Data Base report on osteosarcoma of the head and neck. Cancer 98 (8): 1670-80, 2003. [PUBMED Abstract]
Fernandes R, Nikitakis NG, Pazoki A, et al.: Osteogenic sarcoma of the jaw: a 10-year experience. J Oral Maxillofac Surg 65 (7): 1286-91, 2007. [PUBMED Abstract]
Smeele LE, Kostense PJ, van der Waal I, et al.: Effect of chemotherapy on survival of craniofacial osteosarcoma: a systematic review of 201 patients. J Clin Oncol 15 (1): 363-7, 1997. [PUBMED Abstract]
Ha PK, Eisele DW, Frassica FJ, et al.: Osteosarcoma of the head and neck: a review of the Johns Hopkins experience. Laryngoscope 109 (6): 964-9, 1999. [PUBMED Abstract]
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Canadian Society of Otolaryngology-Head and Neck Surgery Oncology Study Group.: Osteogenic sarcoma of the mandible and maxilla: a Canadian review (1980-2000). J Otolaryngol 33 (3): 139-44, 2004. [PUBMED Abstract]
Guadagnolo BA, Zagars GK, Raymond AK, et al.: Osteosarcoma of the jaw/craniofacial region: outcomes after multimodality treatment. Cancer 115 (14): 3262-70, 2009. [PUBMED Abstract]
McHugh JB, Thomas DG, Herman JM, et al.: Primary versus radiation-associated craniofacial osteosarcoma: Biologic and clinicopathologic comparisons. Cancer 107 (3): 554-62, 2006. [PUBMED Abstract]
Goldstein-Jackson SY, Gosheger G, Delling G, et al.: Extraskeletal osteosarcoma has a favourable prognosis when treated like conventional osteosarcoma. J Cancer Res Clin Oncol 131 (8): 520-6, 2005. [PUBMED Abstract]
Kaste SC, Pratt CB, Cain AM, et al.: Metastases detected at the time of diagnosis of primary pediatric extremity osteosarcoma at diagnosis: imaging features. Cancer 86 (8): 1602-8, 1999. [PUBMED Abstract]
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Bacci G, Mercuri M, Longhi A, et al.: Grade of chemotherapy-induced necrosis as a predictor of local and systemic control in 881 patients with non-metastatic osteosarcoma of the extremities treated with neoadjuvant chemotherapy in a single institution. Eur J Cancer 41 (14): 2079-85, 2005. [PUBMED Abstract]
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