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Bone Cancer

Bone Cancer, Chondrosarcoma and Ewing Sarcoma, Symptoms and Causes

Bone cancer, or primary bone cancer, is cancer that begins in your bones. Primary bone cancer is rare, and different from secondary bone cancer, which starts in other parts of the body and later spreads to the bones. Almost all types of cancer can spread to bone. This section deals exclusively with primary bone cancer.

At the North Shore-LIJ Cancer Institute, individuals with bone cancers are treated by a team of experts that includes specialists in medical oncology, surgical oncology, radiation medicine, pathology, radiology and rehabilitation. Our institute strives to provide the most progressive and comprehensive care to treat, educate and support patients with bone cancer.

The more common types of bone cancer include chondrosarcoma, Ewing Sarcoma Family of Tumors, and osteosarcoma.

Chondrosarcoma: An Overview

What Is Chondrosarcoma?

Chondrosarcoma is a type of cancer that develops in cartilage cells. Cartilage is the specialized, gristly connective tissue that is present in adults and the tissue from which most bones develop. Cartilage plays an important role in the growth process. There are many different types of cartilage that are present throughout the body. Chondrosarcoma is a malignant type of bone cancer that primarily affects the cartilage cells of the femur (thighbone), arm, pelvis, knee and spine. Although less frequent, other areas such as the ribs may be affected.

Chondrosarcoma is the second most common type of primary bone cancer. A primary bone cancer is one that originates from bone, as opposed to starting in another organ and then spreading to the bone. This type of cancer rarely affects individuals under age 20 and continues to rise until age 75. The incidence between males and females is equal.

What Causes Chondrosarcoma?

The exact cause of chondrosarcoma is not known. There may be a genetic or chromosomal component that predisposes certain individuals to this type of malignancy.

What Are the Risk Factors of Chondrosarcoma?

Most often, chondrosarcoma occurs from normal cartilage cells, but it may also stem from a pre-existing benign (non-cancerous) bone or cartilage tumor. The following is a list of some benign conditions that may be present when chondrosarcoma occurs:

  • Enchondromas — a type of benign bone tumor that originates from cartilage and usually affects the hands and can also affect other areas
  • Osteochondromas — an overgrowth of cartilage and bone near the end of the bone near the growth plate
  • Multiple Exostoses — the presence of multiple osteochondromas (an overgrowth of cartilage and bone near the end of the growth plate)
  • Ollier's Disease — a cluster of enchondromas (benign cartilage tumor that usually affects the hands)
  • Maffucci's Syndrome — a combination of multiple enchondromas (benign cartilage tumors that usually affect the hands) tumors and angiomas (benign tumors made up of blood vessels)

What Are the Symptoms of Chondrosarcoma?

Symptoms of chondrosarcoma may vary depending on the location of the tumor. The following are the most common symptoms of chondrosarcoma. However, each individual may experience symptoms differently. Symptoms may include:

  • Large mass on the affected bone
  • Feeling of pressure around the mass
  • Pain that is usually worse at night and may be relieved by taking anti-inflammatory medications such as ibuprofen
  • Pain that is not usually relieved through rest
  • Pain that may be present for years but increases gradually over time

How Is Chondrosarcoma Diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for chondrosarcoma may include the following:

  • Biopsy — a procedure in which tissue samples are removed (with a needle or during surgery) from the body for examination under a microscope to determine if cancer or other abnormal cells are present
  • X-ray — a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs onto film
  • Computed Tomography Scan (also called a CT or CAT scan) — a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat and organs. CT scans are more detailed than general x-rays.
  • Magnetic Resonance Imaging (MRI) — a diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body

How Is Chondrosarcoma Treated?

Specific treatment for chondrosarcoma will be determined by your physician based on:

  • Your age, overall health and medical history
  • Extent of the disease
  • Your tolerance for specific medications, procedures and therapies
  • Expectation for the course of the disease
  • Your opinion or preference

The goal for treatment of chondrosarcoma is to remove the mass and reduce the likelihood that it will return. Close follow-up with your physician may be necessary. Treatment may include:

  • Surgery (to remove the tumor)
  • Physical therapy (to regain strength and use of the affected area after surgery)
  • Chemotherapy

Chemotherapy, although not the primary treatment, may be required if the cancer has spread to other areas of the body.

Ewing Sarcoma: An Overview

What Is Ewing Sarcoma?

Ewing sarcoma is a cancer that occurs primarily in the bone or soft tissue. Ewing sarcoma can occur in any bone, but most often it is found in the long bones such as the femur (thigh), tibia (shin) or humerus (upper arm). It can involve the muscle and the soft tissues around the tumor site as well. Ewing sarcoma cells can also spread (metastasize) to other areas of the body including the bone marrow, lungs, kidneys, heart, adrenal gland and other soft tissues.

Ewing sarcoma accounts for about 2-3 percent of childhood cancers. About 250 children and adolescents will be diagnosed with Ewing sarcoma in the US in 2009. It is the second most common malignant bone tumor in children and adolescents. Ewing sarcoma most often occurs in children between the ages of 10 and 20. More males are affected than females.

What Causes Ewing Sarcoma?

The exact cause of Ewing sarcoma is not fully understood. There seems to be no known risk factors or prevention measures available. However, researchers have discovered chromosomal changes in a cell's DNA that can lead to Ewing sarcoma formation. These changes are not inherited. They develop in children after they are born for no apparent reason.

Some physicians classify Ewing sarcoma as a primitive neuroectodermal tumor (PNET). This means the tumor may have started in fetal, or embryonic, tissue that has developed into nerve tissue.

What Are the Symptoms of Ewing Sarcoma?

The following are the most common symptoms of Ewing sarcoma. However, each individual may experience symptoms differently. Symptoms may include:

  • Pain around the site of the tumor
  • Swelling and/or redness around the site of the tumor
  • Fever
  • Weight loss, decreased appetite
  • Fatigue
  • Paralysis and/or incontinence (if the tumor is in the spinal region)
  • Symptoms related to nerve compression from tumor (e.g., numbness, tingling, paralysis)

The symptoms of Ewing sarcoma may resemble other medical conditions or problems. Always consult your physician for a diagnosis.

How Is Ewing Sarcoma Diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for Ewing sarcoma may include the following:

  • Multiple imaging studies, including the following:
    • X-rays — a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
    • Radionuclide Bone Scans — pictures taken of the bone with a special camera after a slightly radioactive dye has been injected that is absorbed by bone tissue. These are used to detect tumors and bone abnormalities.
    • Magnetic Resonance Imaging (MRI) — a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. This test is done to rule out any associated abnormalities of the nearby soft tissues.
    • Computed Tomography Scan (also called a CT or CAT scan) — a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat and organs. CT scans are more detailed than general x-rays.
    • Positron Emission Tomography (PET) Scan — radioactive-tagged glucose (sugar) is injected into the bloodstream. Tissues that use the glucose more than normal tissues (such as tumors) can be detected by a scanning machine. PET scans can be used to find small tumors or to check if treatment for a known tumor is working.
  • Blood tests (including blood chemistries)
  • Biopsy of the Tumor — a procedure in which tissue samples are removed (with a needle or during surgery) from the body for examination under a microscope; to determine if cancer or other abnormal cells are present; to remove tissue from the affected bone.
  • Bone Marrow Aspiration/Biopsy — a procedure that involves a small amount of bone marrow fluid and tissue to be taken, usually from part of the hip bones, to further examine the number, size and maturity of blood cells and/or abnormal cells; to detect cancer cells

Ewing sarcoma is difficult to distinguish from other similar tumors. Diagnosis is often made by excluding all other common solid tumors and by the use of genetic studies.

Treatment and Prognosis

What Is the Treatment for Ewing Sarcoma?

Specific treatment for Ewing sarcoma will be determined by your physician based on:

  • Your age, overall health and medical history
  • Extent of the disease
  • Your tolerance of specific medications, procedures or therapies
  • Expectations for the course of the disease
  • Your opinion or preference

Treatment may include:

  • Surgery to remove the tumor
  • Chemotherapy
  • Radiation therapy
  • Amputation of the affected arm or leg
  • Resections for metastases (e.g., pulmonary resections of cancer cells in the lung)
  • Rehabilitation, including physical and occupational therapy and psychosocial adaptation
  • Prosthesis fitting and training
  • Supportive care (for the side effects of treatment)
  • Antibiotics (to prevent and treat infections)
  • Continual follow-up care (to determine response to treatment, detect recurrent disease and manage late effects of treatment)

What Is the Prognosis for an Individual with Ewing Sarcoma?

Prognosis for Ewing sarcoma greatly depends on:

  • The extent of the disease
  • The size and location of the tumor
  • Presence or absence of metastasis
  • The tumor's response to therapy
  • Your age and overall health
  • Your tolerance of specific medications, procedures or therapies
  • New developments in treatment

A person who was treated for Ewing sarcoma as a child or adolescent may develop effects months or years after treatment ends. These effects are called late effects. The kind of late effects one develops depends on the location of the tumor and the way it was treated.

Some types of treatment may later affect fertility. If this side effect is permanent, it will cause infertility, or the inability to have children. Both men and women can be affected.

As with any cancer, prognosis and long-term survival can vary greatly from person to person. Every individual is unique, and treatment and prognosis are structured around your needs. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a person diagnosed with Ewing sarcoma. Late effects of radiation and chemotherapy, as well as second malignancies, can occur in survivors of Ewing sarcoma. New methods are continually being discovered to improve treatment and to decrease side effects.

Osteosarcoma: An Overview

What Is Osteosarcoma?

Osteosarcoma is a type of bone cancer that develops in the osteoblast cells that form the outer covering of bone. It occurs most often in children, adolescents and young adults. Approximately 900 new cases of osteosarcoma are reported each year in the U.S. It occurs slightly more often in males than in females and represents more than one-third of all bone tumors.

Osteosarcoma most commonly occurs in the long bones around the knee. Other sites for osteosarcoma include the upper leg, or thighbone, the lower leg, upper arm bone or any bone in the body, including those in the pelvis, shoulder and skull.

Osteosarcoma may metastasize, or spread, into nearby tissues of the foot, or into tendons or muscles. It may also metastasize through the bloodstream to other organs or bones in the body.

What Causes Osteosarcoma?

The exact cause of osteosarcoma is not known, but it is believed to be due to DNA mutations – either inherited or acquired after birth. Other theories and associations have been suggested as risk factors.

What Are the Risk Factors for Osteosarcoma?

Suggested risk factors for osteosarcoma include the following:

  • Teenage growth spurts
  • Being tall for a specific age
  • Previous treatment with radiation for another cancer, especially at a young age or with high doses of radiation
  • Presence of certain benign (non-cancerous) bone diseases
  • Presence of certain rare inherited cancers, such as the following:
    • Li-Fraumeni syndrome — a rare family predisposition to multiple types of cancers (such as soft tissue sarcomas, breast cancer, brain tumors, osteosarcoma, leukemia, melanoma, cancer of the adrenal cortex, and others) caused by a mutation in a gene - the p53 tumor-suppressor gene - that normally curbs cancer.
    • Retinoblastoma – a malignant tumor of the retina that usually occurs in children younger than four years old.

What Are the Symptoms of Osteosarcoma?

The following are the most common symptoms for osteosarcoma. However, each individual may experience symptoms differently. Symptoms may include:

  • Pain in the affected bone
  • Swelling around the affected site
  • Increased pain with activity or lifting
  • Limping
  • Decreased movement of the affected limb

The symptoms of osteosarcoma may resemble other medical conditions such as Ewing sarcoma. Always consult your physician for a diagnosis.

How Is Osteosarcoma Diagnosed?

In addition to a complete medical history and physical examination, diagnostic procedures for osteosarcoma may include the following:

  • Multiple imaging studies of the tumor and sites of possible metastasis, such as:
    • X-ray – a diagnostic test which uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs onto film.
    • Radionuclide Bone Scans — a nuclear imaging method to evaluate any degenerative and/or arthritic changes in the joints; to detect bone diseases and tumors; to determine the cause of bone pain or inflammation. This test is to rule out any infection or fractures.
    • Magnetic Resonance Imaging (MRI) — a diagnostic procedure that uses a combination of large magnets, radiofrequencies and a computer to produce detailed images of organs and structures within the body. This test is done to look for any nearby spread of tumors.
    • Computed Tomography Scan (also called a CT or CAT scan) — a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.
    • Positron Emission Tomography (PET) Scan — Radioactive-tagged glucose (sugar) is injected into the bloodstream. Tissues that use the glucose more than normal tissues (such as tumors) can be detected by a scanning machine. PET scans can be used to find small tumors or to check if treatment for a known tumor is working.
  • Complete blood count (CBC) — a measurement of size, number, and maturity of different blood cells in a specific volume of blood
  • Blood tests (including blood chemistries)
  • Biopsy of the Tumor — a procedure in which tissue samples are removed (with a needle or during surgery) from the body for examination under a microscope; to determine if cancer or other abnormal cells are present; to remove tissue from the affected bone

Treatment and Prognosis

What Is the Treatment for Osteosarcoma?

Specific treatment for osteosarcoma will be determined by your physician based on:

  • Your age, overall health and medical history
  • Extent of the disease
  • Your tolerance for specific medications, procedures or therapies
  • Expectations for the course of the disease
  • Your opinion or preference

Treatment may include:

  • Surgery (i.e., biopsy, resections, bone/skin grafts, limb salvage procedures, reconstructions)
  • Amputation
  • Chemotherapy
  • Radiation therapy
  • Resections of metastases (spreading of the tumor to other locations)
  • Rehabilitation including physical and occupational therapy, and psychosocial adapting
  • Prosthesis fitting and training
  • Supportive care (for the side effects of treatment)
  • Antibiotics (to prevent and treat infections)
  • Continued follow-up care (to determine response to treatment, detect recurrent disease and manage the side effects of treatment)

What is the Prognosis for an Individual with Osteosarcoma?

Prognosis for osteosarcoma greatly depends on:

  • The extent of the disease
  • The size and location of the tumor
  • Presence or absence of metastasis
  • The tumor's response to therapy
  • Your age and overall health
  • Your tolerance of specific medications, procedures or therapies
  • New developments in treatment

A person who was treated for bone cancer as a child or adolescent may develop effects months or years after treatment ends. These effects are called late effects. The kind of late effects one develops depends on the location of the tumor and the way it was treated.

Some types of treatment may later affect fertility. If this side effect is permanent, it will cause infertility, or the inability to have children. Both men and women can be affected.

As with any cancer, prognosis and long-term survival can vary greatly from person to person. Every individual is unique, and treatment and prognosis are structured around your needs. Prompt medical attention and aggressive therapy are important for the best prognosis. Continuous follow-up care is essential for a person diagnosed with osteogenic sarcoma. Side effects of radiation and chemotherapy, as well as second malignancies, can occur in survivors of osteogenic sarcoma. New methods are continually being discovered to improve treatment and to decrease side effects.

Bone Cancer Clinical Trials

The North Shore-LIJ Cancer Institute offers a full array of clinical trials. The result of this research not only impacts survival, but also enhances the quality of life. For more information about clinical trials for Bone Cancer, visit Cancer Clinical Trials.

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