Osteosarcoma

What is osteosarcoma?

Osteosarcoma is a type of bone cancer. In the beginning, the cancer cells appear to be regular bone cells. Then, they create malignant (cancerous) tumors, and those tumors create irregular, diseased bone.

Healthcare providers sometimes call it osteogenic sarcoma. A “sarcoma” is a type of cancer that develops in connective tissue such as bone, cartilage or muscle. “Osteo-” refers to bones. “Osteogenic” means it makes bone cells.

Osteosarcoma most commonly affects kids and teens. Experts estimate that fewer than 1,000 people in the U.S. develop osteosarcoma each year.

Which bones does osteosarcoma affect?

Osteosarcoma most often affects long bones, like the ones in your arms and legs. It usually develops near the ends of your bones around joints, especially near knees, hips or shoulders.

The bones most often affected include the:

• Shin bone (tibia)
• Thigh bone (femur)
• Upper arm bone (humerus)

Other less common locations for osteosarcoma include the:

• Jaw
• Pelvis
• Skull
• Soft tissues or organs in your abdomen (belly) and chest

Osteogenic sarcoma grades

Providers categorize osteogenic sarcomas by three grades depending on how quickly they’ll spread (metastasize):

• Low grade
• Intermediate grade
• High grade

Low-grade tumors grow slowly and usually remain where they form. A high-grade tumor will grow quickly, and is more likely to spread.

What are the symptoms of osteosarcoma?

The most common symptoms of osteosarcoma include:

• Bone pain or tenderness around a bone
• Limited movement in a joint
• A mass or lump (tumor) you can feel (the area around the tumor may feel warm)
• Swelling in one spot on or near a bone
• Discoloration on your skin
• Unexplained fever
• Broken bones that happen suddenly or from injuries that normally wouldn’t cause injuries (pathologic fractures)

How painful is osteosarcoma?

Osteosarcomas can cause intense pain. But some are painless. It depends on where they develop and how fast they grow.

For example, it may be painful to lift things if the tumor is in your arm bones. Or you might limp if it’s in your leg bones.

What causes osteogenic sarcoma?

Experts aren’t certain what causes osteogenic sarcoma. Some causes may include:

• Rapid bone growth. The risk of osteosarcoma increases during growth spurts. That’s why osteogenic sarcoma is most common in kids and teens whose bodies are developing rapidly.
• Exposure to radiation. Exposure to radiation at work, in the environment or as part of radiation therapy to treat other types of cancer may cause osteogenic sarcoma.
• Genetic factors. A genetic change (mutation) that affects your p53 gene may cause some kinds of cancers, including osteosarcoma. P53 is sometimes known as a tumor suppressor gene because it helps your body stop tumors from forming. Experts think osteosarcoma may also be related to the retinoblastoma (Rb) gene, which can be associated with eye cancers in young children.
• Bone infarction. This happens when something cuts off blood supply to your bone tissue. This lack of blood destroys healthy bone cells and may cause cancerous osteogenic sarcoma cells to form.

What are the risk factors?

Teens are the most likely group to develop osteosarcoma. In fact, more than 3 out of every 4 people with osteosarcoma are younger than 25. The average age of diagnosis is 15.

You might have a higher risk if you’ve ever had treatment for another type of cancer. Radiation therapy and taking alkylating agents (certain types of cancer medication) can increase the chances that you develop osteosarcoma in the future.

Having certain health conditions can increase your risk, including:

• Li-Fraumeni syndrome
• Paget’s disease of the bone (osteitis deformans)
• Hereditary retinoblastoma
• Bloom syndrome
• Diamond-Blackfan anemia
• Rothmund-Thomson syndrome
• Werner syndrome

How is osteosarcoma diagnosed?

A healthcare provider will begin with a thorough history and physical examination. They’ll ask about your symptoms and medical history. Tell your provider if you’ve had any prior radiation treatment or if anyone in your biological family has a genetic condition or history of cancer. Your provider will look for lumps that may be protruding from your bones. You’ll probably need to see an oncologist — a healthcare provider who specializes in diagnosing and treating cancer.

Your provider might also use some tests when making a diagnosis, including:

• Biopsy
• Blood tests
• CT scan
• MRI
• X-ray

After your provider diagnoses osteosarcoma, the next step is to find out if the cancer cells have spread. This is called “staging.” The cancer can spread through your blood, your lymphatic system or tissues. You may need additional imaging tests like a whole-body bone scan or PET scan. These tests will help your provider see if the cancer has metastasized to other bones, organs or areas of your body.

What are osteosarcoma treatments?

The most common treatments are chemotherapy (chemo) and surgery to remove osteosarcoma tumors and cancer medications to kill cancer cells throughout your body. You’ll probably need chemo, then surgery, then more chemo.

Your providers might use radiation therapy to treat osteosarcoma, especially if the cancer spreads to areas where you can’t have surgery.

Osteosarcoma surgery

You’ll probably need surgery to remove the osteosarcoma. Your surgeon will try to preserve as much of your natural bone and tissue as they can. But they’ll remove some of the healthy tissue around the tumor to make sure they’re taking out as many cancer cells as possible.

Some surgeries you may need include:

• Limb salvage surgery
• Amputation
• Rotationplasty (a type of amputation)

If you have limb salvage surgery, your surgeon will replace the removed section of your bones with either an artificial implant (prosthesis) or a bone graft.

You may need a prosthetic limb after amputation or rotationplasty.

Complications/side effects of osteosarcoma treatment

You may experience complications or side effects right away. Some can last for months or even years. They might include:

• Changes in thinking, learning or memory
• Developing another type of cancer
• Infertility
• Mood swings
• Neurological problems (issues that affect your brain or nerves)
• Problems with organs including your heart, lungs, ears or kidneys

Can you prevent osteogenic sarcoma?

Because experts aren’t certain why it develops, there’s no way to prevent osteogenic sarcoma. Talk to your healthcare provider about genetic testing for cancer risk if you’re interested in screening yourself or your children. Your provider will tell you if genetic testing is a good idea for you or your family.

What is the survival rate of osteosarcoma?

The survival rate depends on which type of osteosarcoma you have, and if it spreads from its original location.

Experts estimate that 7 in 10 people survive osteosarcoma if it hasn’t metastasized (spread). Survival rates for metastatic (spreading) osteosarcoma are around 5 in 10 people. Your provider will tell you what to expect.

Is osteosarcoma aggressive?

Osteosarcoma can be aggressive. High-grade tumors can spread quickly. Lower-grade osteosarcomas will spread slowly (or not at all).

Cancers metastasize at different rates because everyone’s body and health are unique. Even though experts know that high-grade osteosarcomas can spread quickly, there isn’t a set amount of time that applies equally to everyone.

Two in every 10 cases of metastatic osteosarcoma cases have already spread by the time they’re diagnosed. Visit a healthcare provider as soon as you notice any symptoms or changes in your body that make you feel worried or concerned.

How do I take care of myself?

Maintaining or reclaiming a good quality of life is vital before, during and after treatment for osteogenic sarcoma. It’s not only about taking care of your physical health: your mental and emotional health are just as important. A mental health provider or therapist can help.

When should I see my healthcare provider?

Your healthcare provider will tell you how often you’ll need follow-up visits. They’ll have to check for:

• Signs the cancer is coming back or spreading
• Treatment complications
• Any changes in your health

You’ll need more frequent at first (every three to six months). As you get further from treatment, you might only need one checkup a year. You’ll probably need routine physical exams and lab and imaging tests to monitor any changes in your body.

Which questions should I ask my doctor?

You might want to ask your provider:

• Which grade of osteosarcoma do I have?
• Which type is it?
• Has it spread?
• What’s the best treatment?
• Which type of surgery will I need?
• Which side effects from treatment should I prepare for?