What is Osteosarcoma?

Osteosarcoma is the most common type of bone cancer, which begins in cells that form bones. It is a very rare condition and can also occur in soft tissue outside the bone. Osteosarcoma is most often found in the long bones — more often the legs, but sometimes the arms — but it can start in any bone.

Osteosarcoma tends to occur in teenagers and young adults, but it can also occur in younger children and older adults. Treatment usually involves chemotherapy and surgery. Typically radiation therapy is not effective in treating osteosarcoma, though the use of new improved radiation techniques, such as proton beam therapy, is being studied.

What Causes Osteosarcoma?

The cause of osteosarcoma is not clear and most cases appear to occur sporadically, but some factors may increase the risk. Previous treatment with radiation therapy can increase the risk of developing osteosarcoma. Certain inherited or genetic conditions, including hereditary retinoblastoma, Bloom syndrome, Li-Fraumeni syndrome, Rothmund-Thomson syndrome, Diamond-Blackfan anemia, Paget’s disease and Werner syndrome have been shown to increase the risk of osteosarcoma.

Symptoms

Signs and symptoms of osteosarcoma may include swelling near a bone, bone or joint pain and bone injury or bone break for no clear reason. Because these symptoms can be mistaken for other health issues, such as a sports injury, sometimes there is a delay in seeking medical help.

Treatment

Treatment for osteosarcoma has greatly improved over the years. The outlook (prognosis) and treatment decisions depend on where the osteosarcoma starts, tumor size, the type and grade of osteosarcoma, and whether the cancer has spread. Treatment typically includes limb-sparing surgery and chemotherapy, but there are other options.

The goal of surgery is to remove the cancer cells, but surgeons also perform operations to maintain function and minimize disability. The extent of surgery for osteosarcoma depends on several factors, such as the tumor size, location and the person’s age. There are limb-sparing surgeries for osteosarcoma which focus on removing the tumor and sparing the limb so that function can be maintained. Whether this is feasible depends, in part, on the extent of the cancer and how much muscle and tissue need to be removed.

With advancements in limb-sparing surgery, the need for amputation or removing a limb or part of a limb  has greatly reduced over the years. If amputation is necessary, advances in prosthetic joints can significantly improve outcomes and function.

There is another surgery called rotationplasty. In this surgery which is sometimes used for children who are still growing, the surgeon removes the tumor and surrounding area, including the knee joint. The foot and ankle are then rotated and the ankle functions as a knee. A prosthesis is used for the lower leg and foot. Results typically enable the person to function very well in physical activities, sports and daily living.

Chemotherapy uses drugs to kill cancer cells. Doctors often recommend using chemotherapy before surgery for osteosarcoma, to reduce the size of the tumor, and after surgery, to kill any cancer cells that may remain.

Radiation therapy uses high-energy beams, such as X-rays and protons, to kill cancer cells. Though typically,  radiation is not used due to lack of effectiveness in treating osteosarcoma, newer forms of radiation therapy are being studied and may rarely be used.

Complications

Osteosarcoma can spread from where it started to other areas, making treatment and recovery more difficult. Surgery that removes the tumor and spares the limb is most often the norm. But certain patients may require amputation of the affected part of the limb and then need to learn to use an artificial limb (prosthesis).

As with other types of serious cancer, aggressive chemotherapy for osteosarcoma can cause substantial side effects, both in the short and long term. The health care team takes steps to treat and manage these effects as best as possible. And it’s important for you to learn what to watch for and contact your team with any concerns.

Prevention

There is no known way to prevent osteosarcoma, though certain factors, such as past radiation therapy or certain genetic conditions increase the risk. Still, having a risk factor doesn’t necessarily mean that you’ll get osteosarcoma. But any signs or symptoms should be checked out as soon as possible.

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