Osteosarcoma - Childhood and Adolescence: Risk Factors

Approved by the Cancer.Net Editorial Board, 11/2020

ON THIS PAGE: You will find out more about the factors that increase the chance of developing osteosarcoma. Use the menu to see other pages.

A risk factor is anything that increases a person’s chance of developing cancer. Although risk factors often influence the development of a tumor, most do not directly cause cancer. Some people with several risk factors never develop a tumor, while others with no known risk factors do.

Most osteosarcoma begins sporadically. This means it appears in people who have no other diseases and no family history of bone cancer.

  • Genetic changes. Osteosarcoma is probably caused by a combination of genetic changes, or mutations. Together, these changes cause immature bone cells to become tumor cells instead of healthy bone. Osteosarcoma may start because of overactive bone cells. Researchers are studying possible changes in certain genes that lead to this overactivity. They are studying this in a small number of families in which siblings have developed osteosarcoma. Such changes in genes are uncommon. However, it may help doctors better understand how osteosarcoma begins in people with no family history of the disease, too. Similar genetic changes may occur in their cancer cells.

    A gene known as RB may be associated with osteosarcoma. This gene is abnormal in most children with the hereditary form of a rare type of eye cancer called retinoblastoma. RB is a tumor-suppressor gene that controls cell growth. When it is changed, it no longer controls cell growth. As a result, a tumor can form. Mutations in the p53 gene are referred to as Li-Fraumeni syndrome (see below), which is the most common cancer predisposition syndrome associated with osteosarcoma.

    Many other changes occur in the genes of osteosarcoma cells.

It is rare when anyone is diagnosed with osteosarcoma. However, some groups of people are more likely to develop osteosarcoma:

  • People who have received radiation treatment for another type of cancer. Those who have previously received radiation treatment are more likely to develop osteosarcoma. High doses of radiation treatment at a younger age increase the risk. However, the time between radiation treatment and osteosarcoma risk is typically more than a decade and is rarely the cause of osteosarcoma in children and adolescents.

  • Children with an inherited form of retinoblastoma. This group of children, who most often have changes in the RB gene throughout their body,  are more likely to develop osteosarcoma.

  • Children with Li-Fraumeni syndrome. A child with Li-Fraumeni syndrome has a higher risk of sarcoma, including osteosarcoma, as well as brain cancer, breast cancer, leukemia, and adrenal cancer. Li-Fraumeni syndrome is a rare disorder of the p53 gene. That gene is responsible for getting rid of abnormal cells.

  • Children with Werner syndrome. These children have a higher risk of sarcoma, thyroid cancer, and melanoma. Werner syndrome is a very rare disorder that may involve the WRN gene.

  • People with Rothmund-Thomson syndrome. This group of people is more likely to develop osteosarcoma. Rothmund-Thomson syndrome is an uncommon disorder. It is characterized by short height, rash, hair loss, and skeletal dysplasia. Skeletal dysplasia are noncancerous abnormalities of the bones.

The next section in this guide is Symptoms and Signs. It explains what body changes or medical problems osteosarcoma can cause. Use the menu to choose a different section to read in this guide.