The providers at UofL Health – Brown Cancer Center bring together a team of experts that includes specialists from many areas to give personal, customized care for osteosarcoma, or bone cancer. We use specialized therapies and technologies to be sure you receive the most advanced treatment with the least impact on your body.

As a leading cancer center, we constantly work to discover new treatments and innovations.

It is best to have a biopsy to diagnose bone cancer done at the same place you receive treatment. It is essential to go to a specialized cancer center that has experience in osteosarcoma biopsy. If the biopsy is done incorrectly, it may make it more difficult later for the surgeon to remove all of the cancer without also having to remove all or part of the arm or leg. A biopsy that is not done correctly also may cause the cancer to spread.

Facts

Understanding a disease is the first step toward finding the right care. Get the facts about bone cancer, including the different types, how it starts and who’s at risk.

Bone cancer can develop in the bones or spread to bone from other areas of the body.

According to the American Cancer Society, more than 2,600 people each year in this country are diagnosed with primary cancer of the bones and joints. These cases make up 0.2 percent of all cancers in the United States.

Bone cancer is a sarcoma (type of cancerous tumor) that starts in the bone. Other cancers may affect the bones, including:

  • Secondary cancers that metastasize, or spread, from other parts of the body
  • Other types of cancer, including non-Hodgkin's lymphoma and multiple myeloma

The information on this website is about cancers that start in the bones (primary bone cancer).

Bones support and give structure to the body. They usually are hollow. The main parts of the bones are:

Matrix is the outer part of bones. It is made of fiber-like tissue and is covered with a layer of tissue called the periosteum.

Bone marrow is the soft tissue in the space in hollow bones called the medullary cavity. Cells inside bone marrow include:

  • Fat cells
  • Red blood cells, white blood cells and platelets
  • Fibroblasts, a type of cell that helps build connective tissue
  • Plasma, in which blood cells are suspended

Cartilage is at the end of most bones. It is softer than bone, but it is firmer than soft tissue. Cartilage and other tissues, including ligaments, make up joints that connect some bones.

Bones constantly change as new bone forms and old bone dissolves. To make new bone, the body deposits calcium into the cartilage. Some of the cartilage stays at the ends of bones to make joints.

Bone cancer types

There are several types of bone tumors. They are named according to the area of bone or tissue where they start and the type of cells they contain. Some bone tumors are benign (not cancer), and some are malignant (cancer). Bone cancer also is called sarcoma.

The most commonly found types of primary bone cancer are:

Osteosarcoma or osteogenic sarcoma is the main type of bone cancer. It occurs most often in children and adolescents, and it accounts for about one-fourth of bone cancer in adults. More males than females get this cancer. About 1,000 people in the United States are diagnosed with osteosarcoma each year. It begins in bone cells, usually in the pelvis, arms or legs, especially the area around the knee.

Chondrosarcoma is cancer of cartilage cells. More than 40 percent of adult bone cancer is chondrosarcoma, making it the most prevalent bone cancer in adults. The average age of diagnosis is 51, and 70 percent of cases are in patients older than 40. Chondrosarcoma tends to be diagnosed at an early stage and often is low grade. Many chondrosarcoma tumors are benign (not cancer). Tumors can develop anywhere in the body where there is cartilage, especially the pelvis, leg or arm.

Ewing's sarcoma is the second most prevalent type of bone cancer in children and adolescents, and the third most often found in adults. It accounts for about 8 percent of bone cancers in adults. Ewing's sarcoma can start in bones, tissues or organs, especially the pelvis, chest wall, legs or arms.

Less-commonly found types of bone cancer include:

  • Chordoma, which is found in 10% of adult bone cancer cases, usually in the spine and base of the skull
  • Malignant fibrous histiocytoma/fibrosarcoma, which usually starts in connective tissue
  • Fibrosarcoma, which often is benign and found in soft tissue in the leg, arm or jaw

Secondary (or metastatic) bone cancer is cancer that spreads to the bone from another part of the body. This type of bone cancer is more prevalent than primary bone cancer. For more information about this type of cancer, see the type of primary cancer (where the cancer started).

Risk factors

Anything that increases your chance of getting bone cancer is a risk factor. However, having risk factors does not mean you will get bone cancer. In fact, most people who develop bone cancer do not have any risk factors. If you have risk factors, it’s a good idea to discuss them with your health care provider.

Teenagers and young adults are at greatest risk of developing osteosarcoma, a type of bone cancer, because it often is associated with growth spurts.

Some diseases that run in families can slightly increase the risk of bone cancer. Genetic counseling may be right for you. These include:

  • Li-Fraumeni syndrome
  • Rothmund-Thompson syndrome
  • Retinoblastoma (an eye cancer of children)
  • Multiple osteochondromas

Other risk factors for bone cancer include:

  • Paget’s disease
  • Prior radiation therapy for cancer, especially treatment at a young age or with high doses of radiation
  • Bone marrow transplant

Symptoms

Most cancers have the same symptoms as other, less serious conditions. Still, it’s important to know the signs.

Bone cancer symptoms vary from person to person. They also depend on the size and location of the cancer.

If you have symptoms of bone cancer, they may include:

  • Pain
  • Swelling or tenderness in or near a joint
  • Difficulty with normal movement
  • Fatigue
  • Fever
  • Weight loss
  • Anemia (low red blood cell count)
  • Fractures

Having one or more of these symptoms does not mean you have bone cancer. However, it is important to discuss any symptoms with your doctor, since they may indicate other health problems.

Diagnosis

Blood tests, imaging exams and even surgical procedures are used to check for cancer.

Accurate diagnosis is essential to the successful treatment of bone cancer. The wrong kind of biopsy may make it more difficult later for the surgeon to remove all of the cancer without having to also remove all or part of the arm or leg. A biopsy that is not done correctly may cause the cancer to spread.

If your doctor thinks you may have bone cancer, it’s important to go to a cancer center with a specialized bone cancer program. You should look for a program that does as many diagnostic procedures as possible.

If you have symptoms that may signal bone cancer, your doctor will examine you and ask you questions about your health and your family history. One or more of the following tests may be used to find out if you have cancer and if it has spread. These tests also may be used to find out if treatment is working.

Biopsy

A biopsy, which removes a tiny piece of bone, is used to confirm the presence of cancer cells. This is the only way to find out for certain if the tumor is cancer or another bone disease. It is very important for the biopsy procedure to be done by a surgeon with experience in diagnosing and treating bone tumors.

There are two types of bone biopsy:

Needle biopsy: A long, hollow needle is inserted through the skin to the area of bone to be tested. The needle removes a cylindrical sample of bone to look at under a microscope.

Open or surgical biopsy: An incision (cut) is made, and the surgeon removes a tiny piece of bone for examination under a microscope.

Your doctor will decide which type of biopsy is best for you based on several factors, including the type and location of the tumor. If possible, the surgeon who performs the biopsy should also do the surgery to remove the cancer.

Bone cancer staging

If you are diagnosed with bone cancer, your doctor will determine the stage (or extent) of the disease. Staging is a way of determining how much disease is in the body and where it has spread.

This information is important because it helps your doctor determine the best type of treatment for you and the outlook for your recovery (prognosis). Once the staging classification is determined, it stays the same even if treatment is successful or the cancer spreads.

One system that is used to stage all bone cancer is the American Joint Commission on Cancer (AJCC) system. (source: National Cancer Institute)

  • T stands for features of tumor (its size)
  • N stands for spread to lymph nodes
  • M is for metastasis (spread) to distant organs
  • G is for the grade of the tumor

This information about the tumor, lymph nodes, metastasis and grade is combined in a process called stage grouping.

The stage is then described in Roman numerals from I to IV (1-4).

T stages of bone cancer:

  • TX: Primary tumor can't be measured
  • T0: No evidence of the tumor
  • T1: Tumor is eight centimeters (around three inches) or less
  • T2: Tumor is larger than eight centimeters
  • T3: Tumor is in more than one place on the same bone

N stages of bone cancer:

  • N0: The cancer has not spread to the lymph nodes near the tumor
  • N1: The cancer has spread to nearby lymph nodes

M stages of bone cancer:

  • M0: The cancer has not spread anywhere outside of the bone or nearby lymph nodes
  • M1: Distant metastasis (the cancer has spread)
  • M1a: The cancer has spread only to the lung
  • M1b: The cancer has spread to other sites (like the brain, the liver, etc)

Grades of bone cancer:

  • G1-G2: Low grade
  • G3-G4: High grade

TNM stage grouping

After the T, N and M stages and the grade of the bone cancer have been determined, the information is combined and expressed as an overall stage. The process of assigning a stage number is called stage grouping.

To determine the grouped stage of a cancer using the AJCC system, find the stage number below that contains the T, N and M stages and the proper grade.

Stage I: All stage I tumors are low grade and have not yet spread outside of the bone.

  • Stage IA: T1, N0, M0, G1-G2: The tumor is eight centimeters or less.
  • Stage IB: T2 or T3, N0, M0, G1-G2: The tumor is either larger than eight centimeters or it is in more than one place on the same bone.

Stage II: Stage II tumors have not spread outside the bone (like stage I) but are high grade.

  • Stage IIA: T1, N0, M0, G3-G4: The tumor is eight centimeters or less.
  • Stage IIB: T2, N0, M0, G3-G4: The tumor is larger than eight centimeters.

Stage III: T3, N0, M0, G3-G4: Stage III tumors have not spread outside the bone but are in more than one place on the same bone. They are high grade.

Stage IV: Stage IV tumors have spread outside of the bone they started in. They can be any grade.

  • Stage IVA: Any T, N0, M1a, G1-G4: The tumor has spread to the lung.
  • Stage IVB: Any T, N1, any M, G1-G4 OR Any T, any N, M1b, G1-G4: The tumor has spread to nearby lymph nodes or to distant sites other than the lung (or both).

Even though the AJCC staging system is widely accepted and used for most cancers, bone cancer specialists tend to simplify the stages into localized and metastatic. Localized includes stages I, II and III, while metastatic is stage IV.

Treatment

Common cancer treatments include chemotherapy, radiation treatment and surgery. Doctors select the treatment for bone cancer based on your diagnosis and disease stage.

If you are diagnosed with bone cancer, your doctor will discuss the best options to treat it. This depends on several factors, including the type and stage of the cancer and your general health.

Your treatment for bone cancer will be customized to your particular needs.

One or more of the following therapies may be recommended to treat bone cancer or help relieve symptoms.

Surgery

Surgery is the main treatment for most bone cancers. Both the biopsy and surgery should be done by a surgeon with extensive experience in these procedures. A biopsy in the wrong location can cause surgical problems and lower your chances of successful treatment.

If at all possible, the same surgeon should perform both the biopsy and surgery. The biopsy will help the surgeon locate the tumor more precisely. The goal of surgery is to remove as much of the cancer as possible. If any cancer cells remain, they may grow and spread. To get as much of the cancer as possible, the surgeon performs a wide-excision surgery. This involves removing the cancer, as well as a margin of healthy tissue around it.

If the tumor is in an arm or leg, the surgeon almost always is able to perform limb-sparing surgery, which removes the cancer cells but allows you to keep full use of your leg or arm. To replace bone that is removed during surgery, a bone graft may be done or an internal device called an endoprosthesis may be implanted.

If this is not possible, an amputation, or removal of the limb, may be performed. Reconstructive surgery and/or a prosthesis will be needed. Rehabilitation is necessary after either procedure.

Chemotherapy

Chemotherapy may be recommended to treat osteosarcoma or Ewing’s sarcoma. In osteosarcoma, it is often given before surgery to shrink the tumor and make it easier to remove, and after surgery to destroy remaining cancer cells. Chemotherapy is also used for bone cancer that has metastasized (spread) to the lungs or other organs.

Radiation therapy

Bone cancer is not highly sensitive to radiation, so radiation usually is not a treatment. It sometimes may be given if the tumor cannot be operated on or if cancer cells remain after surgery. Radiation may help relieve symptoms if bone cancer returns.

New radiation therapy techniques and remarkable skill allow our doctors to target tumors more precisely, delivering the maximum amount of radiation with the least damage to healthy cells.

Proton therapy

Proton therapy delivers high radiation doses directly into the tumor, sparing nearby healthy tissue and vital organs.

Targeted therapy

These newer agents are used to help fight some types of bone cancer, including chordoma. Targeted therapies attack cancer cells by using small molecules to block pathways that cells use to survive and multiply.

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