Bone Cancer

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What is Bone Cancer? 

The human skeletal system is made up of more than 200 bones that protect the internal organs, allow people to stand upright, and attach to muscles that allow movement. Bones are connected to other bones by ligaments, which are bands of tough, fibrous tissue, while cartilage covers and protects the joints where bones come together. Bones are hollow and filled with bone marrow, which is the spongy, red tissue that produces blood cells. The cortex is the hard, outer portion of the bone.

Bone is a tissue that consists of collagen (a soft, fibrous tissue) and calcium phosphate (a mineral that helps harden and strengthen the bone). There are three types of bone cells:

Osteoclasts break down and remove old bone.

Osteoblasts build new bone.

Osteocytes carry nutrients to the bone.

About bone cancer

Cancer can occur in any part of the bone. Cancer begins when normal cells in the bone change and grow uncontrollably, forming a mass called a tumor. A bone tumor can be benign (noncancerous) or malignant (cancerous, meaning it can spread to other parts of the body). Even though a benign tumor does not spread outside the bone,

it can grow large enough to press on surrounding tissue and weaken the bone. A malignant tumor can destroy the cortex and spread to nearby tissue. If bone tumor cells get into the bloodstream, they can spread to other parts of the body, especially the lungs.

There are different types of bone cancer, including:

Osteosarcoma and Ewing sarcoma are two of the most common types of bone cancer and mostly occur in children and young adults.

Chondrosarcoma is cancer of the cartilage and is more common in adults.

Chordoma is a type of bone cancer that typically starts in the lower spinal cord.

Rarely, soft tissue sarcomas begin in the bone, including:

Malignant fibrous histiocytoma (MFH), which makes up less than 1% of bone tumors and is usually found in adults. An arm or leg, especially around the knee joint, is the most common place for MFH to appear.

Fibrosarcoma is also more common among adults, particularly during middle age, and most often begins in the thighbone.

Paget’s disease of the bone generally occurs in older adults and involves the overgrowth of bony tissue.  

Symptoms & Signs

People with bone cancer may experience the following symptoms or signs. Sometimes, people with bone cancer do not show any of these symptoms. Or, these symptoms may be caused by a medical condition that is not cancer. If you are concerned about a symptom or sign described below, please talk with your doctor.

When a bone tumor grows, it presses on the normal tissue and destroys bone tissue, which can cause symptoms. The earliest symptoms of bone cancer are pain and swelling in the area of the tumor. The pain may come and go at first, then become more severe and steady later. The pain may worsen with movement, and there may be swelling in the soft tissue nearby. A tumor that occurs near or in joints may cause the joint to swell and become tender or stiff, which means a person may have a limited and painful range of movement.

A pronounced limp (if the leg is affected) or a fracture (break) in the bone with the tumor are symptoms of later-stage bone cancer. Rarely, people with bone cancer may have symptoms such as fever, generally feeling unwell, weight loss, and anemia (low red blood cell level).

Your doctor will ask you questions about the symptoms you are experiencing to help find out the cause of the problem, called a diagnosis. This may include how long you’ve been experiencing the symptom(s) and how often.

If cancer is diagnosed, relieving symptoms and side effects remains an important part of cancer care and treatment. This may also be called symptom management, palliative care, or supportive care. Be sure to talk with your health care team about symptoms you experience, including any new symptoms or a change in symptoms.

Risk Factors

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors can often influence the development of cancer, most do not directly cause cancer. Some people with several risk factors never develop cancer, while others with no known risk factors do. However, knowing your risk factors and talking about them with your doctor may help you make more informed lifestyle and health care choices.

The following factors may raise a person’s risk of developing bone cancer:

Genetics. Children with familial retinoblastoma (an eye cancer) have an increased risk of developing osteosarcoma.

Previous radiation therapy. People who have had radiation treatment for other conditions have a higher risk of developing bone cancer at the site of the radiation therapy. The majority of radiation therapy-caused bone cancers are osteosarcomas, but other types may occur.

Chemotherapy. Some drugs, including alkylating agents and anthracyclines, used to treat cancer may increase the risk of developing a secondary cancer, usually osteosarcoma.

Benign tumors or other bone conditions. Paget’s disease may cause osteosarcoma. Other noncancerous bone diseases, such as fibrous dysplasia, may increase the risk of osteosarcoma.

Currently, there is no known way to prevent bone cancer. Early detection offers the best hope for successful treatment, so people with risk factors are encouraged to visit the doctor regularly and talk with him or her about it. Still, most bone cancer occurs in people with no known risk factors.

Diagnosis

Doctors use many tests to diagnose cancer and find out if it has metastasized (spread). Some tests may also determine which treatments may be the most effective. For most types of cancer, a biopsy is the only way to make a definitive diagnosis of cancer. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis.

Imaging tests, such as an x-ray, may be used to find out whether the cancer has metastasized. Benign and cancerous tumors usually look different on imaging tests (see below). A benign tumor has round, smooth, well-defined borders. A cancerous tumor has irregular, poorly defined border because of aggressive growth. There may also be evidence of bone destruction on an image of a cancerous tumor. Imaging tests may suggest a diagnosis of bone cancer, but a biopsy will be performed whenever possible to confirm the diagnosis and find out the subtype. It is extremely important for a patient to be seen by a sarcoma specialist before any surgery or a biopsy is performed.

Your doctor may consider these factors when choosing a diagnostic test:

Age and medical condition

Type of cancer suspected

Severity of symptoms

Previous test results

In addition to a physical examination, the following tests may be used to diagnose bone cancer:

Blood tests. Some laboratory tests may help detect bone cancer. Alkaline phosphatase and lactate dehydrogenase levels in the blood may be higher in patients with osteosarcoma or Ewing’s sarcoma. However, it is important to note that alkaline phosphatase is normally high when cells that form bone tissue are very active (for example, when children are growing or a broken bone is healing), so high levels do not always mean cancer. Abnormal glucose tolerance may be found in people with chondrosarcoma.

X-ray. An x-ray is a way to create a picture of the structures inside of the body using a small amount of radiation.

Bone scan. A bone scan uses a radioactive tracer to look at the inside of the bones. The tracer is injected into a patient’s vein. It collects in areas of the bone and is detected by a special camera. Healthy bone appears gray to the camera, and areas of injury, such as those caused by cancerous cells, appear dark.

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a vein to provide better detail.

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture. MRI scans are used to check for any tumors in nearby soft tissue.

Positron emission tomography (PET) scan. A PET scan is a way to create picture of organs and tissues inside the body. A small amount of a radioactive substance is injected into a patient’s body. This substance is absorbed mainly by organs and tissues that use the most energy. Because cancer tends to use energy actively, it absorbs more of the radioactive substance. A scanner then detects this substance to produce images of the inside of the body.

Integrated PET-CT scan. This scanning method collects images from both CT and PET scans at the same time, and then combines the images. This technique helps the doctor look at both the structure and how energy is used by the tumor and normal tissue. This information can help doctors plan treatment and determine the benefits of different treatments.

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease). The type of biopsy (needle or incisional, see below) performed depends on where the cancer is located. However, sometimes a biopsy may not be able to be performed.

For a needle biopsy, a small hole is made in the bone, and a tissue sample is removed from the tumor with a needle-like instrument. During an incisional biopsy, the tissue sample is removed after a small cut is made in the tumor.

Stages

Staging is a way of describing where the cancer is located, if and where it has spread, and whether it is affecting the functions of other organs in the body. Doctors use diagnostic tests to determine the cancer's stage, so staging may not be complete until all of the tests are finished. Knowing the stage helps the doctor to decide what kind of treatment is best and can help predict a patient's prognosis (chance of recovery). There are different stage descriptions for different types of cancer.

One tool that doctors use to describe the stage is the TNM system. This system judges three factors: the tumor itself, the lymph nodes around the tumor, and if the tumor has spread to the rest of the body. The results are combined to determine the stage of cancer for each person. There are five stages: stage 0 (zero) and stages I through IV (one through four). The stage provides a common way of describing the cancer, so doctors can work together to plan the best treatments.

TNM is an abbreviation for tumor (T), node (N), and metastasis (M). Doctors look at these three factors to determine the stage of cancer:

How large is the primary tumor and where is it located? (Tumor, T)

Has the tumor spread to the lymph nodes? (Node, N)

Has the cancer metastasized to other parts of the body? (Metastasis, M)

Tumor. Using the TNM system, the “T” plus a letter or number (0 to 4) is used to describe the size and location of the tumor. Some stages are also divided into smaller groups that help describe the tumor in even more detail. Specific tumor stage information for bone cancer is listed below.

TX: The primary tumor cannot be evaluated.

T0: There is no evidence of a primary tumor.

T1: The tumor is 8 centimeters (cm) or smaller.

T2: The tumor is larger than 8 cm.

T3: There is more than one separate tumor in the primary bone site.

Node. The “N” in the TNM staging system stands for lymph nodes, the tiny, bean-shaped organs that help fight infection. Lymph nodes near where the cancer started are called regional lymph nodes. Lymph nodes in other parts of the body are called distant lymph nodes. Spread to the regional lymph nodes is rare for primary bone cancer.

NX: The regional lymph nodes cannot be evaluated.

N0: The cancer has not spread to the regional lymph nodes.

N1: The cancer has spread to the regional lymph nodes.

Distant metastasis. The “M” in the TNM system indicates whether the cancer has spread to other parts of the body.

MX: Metastasis cannot be evaluated.

M0: The cancer has not metastasized.

M1: There is metastasis to another part of the body.

M1a: There is metastasis to the lung.

M1b: There is metastasis to another organ.

Grade. A cancer may also be graded. The grade describes how much cancer cells look like healthy cells under a microscope. If they look like healthy cells, called well differentiated, the cancer is a low-grade tumor. If they look very little like healthy cells, called poorly differentiated, the cancer is a high-grade tumor. A tumor’s grade is described using the letter “G” and a number. The grade of cancer can help the doctor predict how quickly the cancer will spread. In general, the lower the grade, the better the prognosis.

GX: The tumor grade cannot be identified.

G1: The cancer cells are well differentiated.

G2: The cancer cells are moderately differentiated.

G3: The cancer cells are poorly differentiated.

G4: The cancer cells are undifferentiated.

Cancer stage grouping

Doctors assign the stage of the cancer by combining the T, N, M, and G classifications. In general, patients with the best prognosis have:

T1 or T2 tumor

A lower grade tumor (G1 or G2)

A tumor that is easily removed with surgery, such as those located in an arm or leg

A localized tumor that has not spread

Certain genetic changes

Stage IA: The tumor is low grade (G1 or G2) and 8 cm or smaller (T1). It has not spread to any lymph nodes or other parts of the body (N0, M0).

Stage IB: The tumor is low grade (G1 or G2) and larger than 8 cm (T2). It has not spread to any lymph nodes or other parts of the body (N0, M0).

Stage IIA: The tumor is high grade (G3 or G4) and 8 cm or smaller (T1). It has not spread to any lymph nodes or other parts of the body (N0, M0).

Stage IIB: The tumor is high grade (G3 or G4) and larger than 8 cm (T2). It has not spread to any lymph nodes or other parts of the body (N0, M0).

Stage III: There are multiple high grade (G3 or G4) tumors in the primary bone site (T3), but they have not spread to any lymph nodes or other parts of the body (N0, M0).

Stage IVA: The tumor is of any size or grade and has spread to the lung(s) (any G, any T, N0, and M1a).

Stage IVB: The tumor is of any size or grade and has spread to the lymph nodes (any G, any T, N1, and any M), or the tumor is of any size or grade and has spread to another organ (any G, any T, any N, and M1b).

Recurrent: Recurrent cancer is cancer that comes back after treatment. If there is a recurrence, the cancer may need to be staged again (called re-staging) using the system above.

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