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(A-Z listing includes diseases, conditions, tests and procedures)
 

Osteosarcoma

Osteosarcoma is a type of bone cancer that usually develops in the osteoblast cells that form bone. It happens most often in children, adolescents and young adults. Approximately 1,000 new cases of osteosarcoma are reported each year in the U.S. Of these cases, about 450 involve children and teens. It happens slightly more often in males than in females. It often develops during the period of rapid growth that occurs during adolescence. The average age at diagnosis is 15 years.

Osteosarcoma most commonly happens in the long bones around the knee. Other sites for osteosarcoma include the upper leg (thighbone), the lower leg, upper arm bone or any bone in the body. Additional locations may include bones in the pelvis, shoulder and skull.

Osteosarcoma may grow into nearby tissues, such as tendons or muscles. It may also metastasize (spread) through the bloodstream to other organs or bones in the body.

Osteosarcoma | Jaliyah's Story

After 9-year-old Jaliyah was diagnosed with osteosarcoma, her mother searched for an option to save her leg from amputation. Carol Morris, M.D., M.S., offered an alternative — a complex limb-sparing surgery, which allowed Jaliyah to continue dancing.

Osteosarcoma Subtypes

There are several subtypes of osteosarcomas that vary in the following ways:

  • Appearance under the microscope

  • Rate of growth

  • Likelihood of metastasis

Low-Grade Osteosarcoma

Parosteal osteosarcoma is low grade and grows on the surface of the bone. As the most common type of surface osteosarcoma, this slow-growing cancer typically affects portions of the femur (thighbone), tibia (shinbone) and humerus (upper arm bone). The average age of patients with this condition is 28 years.

Low-grade central osteosarcoma (also known as intramedullary or intraosseous well-differentiated osteosarcoma) is a rare, well-differentiated osteosarcoma. Well-differentiated cancer looks similar to normal cells and tends to grow and spread more slowly than poorly differentiated cancer. Patients typically develop low-grade central osteosarcoma during their thirties. This condition usually affects the long bones, including the femur and tibia.

Intermediate-Grade Osteosarcoma

Periosteal osteosarcoma is very rare and also occurs on the surface of the bone. It usually grows on the tibia and femur. Like parosteal osteosarcoma, the average age of patients with periosteal osteosarcoma is around 28 years.

High-Grade Osteosarcoma

High-grade osteosarcoma is the fastest-growing form of osteosarcoma. According to the American Cancer Society, most osteosarcomas that develop in children and teens are considered high grade.

There are several types of high-grade osteosarcomas. For example, telangiectatic osteosarcoma starts in the bone and can break through into the surrounding tissues. The tumor is filled with large blood-filled spaces and has a distinctive appearance on X-rays and under the microscope.

Some high-grade osteosarcomas develop as a result of patients receiving prior radiation for a different cancer. Patients with Paget’s disease are also at higher risk of developing osteosarcoma.

Other Bone Tumors

The following bone tumors usually occur in adults and are rarely found in children:

  • Chondrosarcoma

  • Malignant fibrous histiocytoma (MFH)

  • Fibrosarcoma

  • Chordoma

Bone Sarcomas | FAQ with Dr. Adam Levin

Orthopaedic oncologist Adam Levin answers questions about the types of bone sarcomas, their diagnosis and available treatments. He highlights the importance of the timing of different treatment procedures to ensure the best results.

Osteosarcoma Causes

While the exact cause of osteosarcoma is unknown, medical researchers and doctors believe that it may be due to DNA mutations that occur inside the bone cells—either inherited or acquired after birth.

Osteosarcoma Risk Factors

The most common 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 (noncancerous) bone diseases

  • Presence of certain rare, inherited disorders, such as the following:

    • Li-Fraumeni syndrome. A rare family predisposition to multiple types of cancers, including soft tissue sarcomas, breast cancer, brain tumors and osteosarcoma. This condition is caused by a mutation in the p53 tumor-suppressor gene, which normally curbs cancer growth.

    • Rothmund-Thomson syndrome. A rare, inherited syndrome that includes skeletal problems, rashes, short stature and an increased risk of developing osteosarcoma. It is caused by an abnormality in the RECQL4 gene.  

    • Hereditary retinoblastoma. A cancer of the eye that usually happens in children under age 4.

Osteosarcoma Symptoms

Patients may experience symptoms differently. The following are the most common symptoms of osteosarcoma:

  • 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. Always consult your health care provider for a diagnosis.

Osteosarcoma Diagnosis

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

  • Multiple imaging tests of the tumor and sites of possible metastasis, such as:

    • X-rays. This diagnostic test uses invisible electromagnetic energy beams to produce images of internal tissues, bones and organs on film.

    • Bone scans. This nuclear imaging method is used to evaluate any degenerative and/or arthritic changes in the joints, to detect bone diseases and tumors and to determine the cause of bone pain or inflammation.

    • Magnetic resonance imaging (MRI). This imaging technique 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 better define a mass seen on an X-ray and to look for any nearby spread of tumors.

    • Computed tomography (CT or CAT) scan. This imaging test uses X-rays and a computer to make detailed images of the body. A CT scan shows details of the bones, muscles, fat and organs. 

    • 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 that have spread or to verify that a treatment for a known tumor is working.

  • Complete blood count (CBC) test. This test measures the size, number and maturity of different blood cells in a specific volume of blood.

  • Other blood tests. These might include tests that analyze various blood chemistries.

  • Biopsy of the tumor. This test involves removing tissue samples (with a needle or during surgery) from the body and examining them under a microscope. The pathologist checks to see if cancer or other abnormal cells are present.

More Information About Osteosarcoma from Johns Hopkins Medicine

Maya meeting with Dr. Morris

When Rotationplasty Is the Right Fit

At age 9, Maya was diagnosed with bone cancer in her femur. Her treatment options were an above-knee amputation, limb salvage with an internal prosthesis or a more unconventional approach: rotationplasty, an unusual operation that allows the ankle to serve as a knee. Carol Morris, chief of Johns Hopkins’ Division of Orthopaedic Oncology, is one of a select group of surgeons in the United States who perform this alternative reconstructive procedure.

Read the article.

Osteosarcoma Treatment

Specific treatment for osteosarcoma will be determined by your health care provider based on the following:

  • Your age, overall health and medical history

  • The type, stage (extent) and location of the osteosarcoma 

  • Your tolerance to specific medicines, procedures or therapies

  • The expectations for the course of the disease

  • Your opinion or preference

Osteosarcoma Treatment Options

Treatment may include the following:

  • Surgery (e.g., biopsies, resections, bone/skin grafts, limb salvage procedures, reconstructions or amputations)

  • Chemotherapy (may be used before or after surgery to reduce the risk of recurrence)

  • Radiation therapy (useful when the tumor can’t be completely removed through surgery; also may be used to slow tumor growth and control symptoms)

  • Rehabilitation, including physical therapy, occupational therapy and psychosocial adaptation

  • Prosthetic fitting and training

  • Supportive care for the side effects of treatment

  • Antibiotics to prevent and treat infections

  • Continued follow-up care to gauge your response to treatment, find recurrent disease and manage the side effects of treatment

Many patients, particularly those with higher grade tumors, will receive a combination of treatments. For most patients, both chemotherapy and surgery are used to treat osteosarcoma.

Osteosarcoma Treatment in Children

For children and teens with osteosarcoma, the treatment team will include the child’s pediatrician as well as children’s cancer specialists. The team will work together to help manage side effects and deal with other problems related to treatment. These issues can be more complex in young cancer patients and require special expertise and management.

Follow-up Care for Osteosarcoma

Follow-up care is vital following osteosarcoma treatment. Your physician will monitor your condition to check for recurrence, watch for metastasis and manage side effects. Your follow-up care plan may include the following:

  • Discussions about symptoms

  • Physical exams

  • Blood tests

  • Imaging tests (CT scans or X-rays)

Some drug therapies may cause hearing problems or heart damage. Your cancer care team will perform tests to check your hearing and heart function.

Physical therapy and rehabilitation may also be used as part of your recovery following osteosarcoma treatment.

More Information About Sarcoma from Johns Hopkins Medicine

Sarcoma Treatment: Answers from Oncologist Carol Morris

A sarcoma diagnosis can be scary. Learn about sarcoma treatment options and prevention of sarcoma recurrence from an expert at the Johns Hopkins Sidney Kimmel Comprehensive Cancer Center.

Read more

Long-Term Outlook for Patients with Osteosarcoma

The prognosis for osteosarcoma patients greatly depends on the following:

  • The extent of the disease

  • The size and location of the tumor

  • The pathologic grade of the cancer 

  • The tumor's response to therapy

  • Your age and overall health

  • Your tolerance to specific medicines, procedures or therapies

  • New developments in treatment

A patient who was treated for bone cancer as a child or adolescent may develop health effects months or years after treatment ends. These are called late effects. The location of the tumor and the way it was treated will determine the type of late effects that occur.

Some treatments may later affect fertility (ability to have children). If this side effect is permanent, it might cause infertility. Both men and women can be affected by infertility following osteosarcoma treatment.

As with any cancer, prognosis and long-term survival can vary greatly from person to person. Every individual is unique. You will receive treatment and prognosis information that is structured to your specific health needs. Prompt medical attention and aggressive therapy help ensure the best prognosis.

Continuous follow-up care is essential for patients diagnosed with osteosarcoma. Side effects of radiation and chemotherapy, including second cancers, can happen in survivors. New methods are continually being discovered to improve treatment and decrease side effects.

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