What is 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 800 new cases of osteosarcoma are reported each year in the U.S. Of these cases, about 400 are in children and teens. It happens slightly more often in males than in females.
Osteosarcoma most commonly happens 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 grow into nearby tissues, such as tendons or muscles. It may also spread, or 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 inside bone cells—either inherited or acquired after birth.
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 (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 (such as soft tissue sarcomas, breast cancer, brain tumors, osteosarcoma, and others) caused by a mutation in a gene—the p53 tumor-suppressor gene—that normally curbs cancer.
Rothmund-Thompson 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 REQL4 gene.
Hereditary retinoblastoma. A cancer of the eye that usually happens in children younger than 4 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
Decreased movement of the affected limb
The symptoms of osteosarcoma may resemble other medical conditions. Always talk with your healthcare provider 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 tests of the tumor and sites of possible metastasis, such as:
X-ray. A diagnostic test that uses invisible electromagnetic energy beams to produce images of internal tissues, bones, and organs on film.
Bone scans. A nuclear imaging method 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). A 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 better define a mass seen on X-ray and to look for any nearby spread of tumors.
Computed tomography scan (also called a CT or CAT scan). This is an imaging test that 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 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.
Other blood tests. These might include blood chemistries.
Biopsy of the tumor. A procedure in which tissue samples are removed (with a needle or during surgery) from the body for exam under a microscope to determine if cancer or other abnormal cells are present.
Treatment for osteosarcoma
Specific treatment for osteosarcoma will be determined by your healthcare provider based on:
Your age, overall health, and medical history
Type, stage (extent), and location of the osteosarcoma
Your tolerance for specific medicines, procedures, or therapies
Expectations for the course of the disease
Your opinion or preference
Treatment may include:
Surgery (for example, biopsy, resections, bone/skin grafts, limb salvage procedures, reconstructions, or amputation)
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, find recurrent disease, and manage the side effects of treatment
Many patients, particularly those with higher grade tumors, will receive a combination of treatments.
Long-term outlook for an individual with osteosarcoma
Prognosis for osteosarcoma greatly depends on:
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 of specific medicines, 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 might 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 is 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 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.
March 21, 2017
Osteosarcoma: Epidemiology, Pathogenesis, Clinical Presentation, Diagnosis, and Histology. UpToDate
Alteri, Rick, MD,Levin, Mark, MD