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Understanding Sarcoma

Risk Factors for Sarcoma

Last updated and reviewed on May 27, 2026.

A risk factor is anything that increases the chance that a person will develop a certain disease. Having one or more risk factors does not mean you will definitely get sarcoma; many people with sarcoma have no known risk factors at all. And many people with known risk factors never develop the disease. Still, knowing the risk factors can help you and your doctor watch for problems and have informed conversations about your health.

What does NOT cause sarcoma: It is important to note that injuries and bumps do not cause sarcoma. Sometimes a person notices a sarcoma after an injury, but the injury did not cause the cancer; the injury simply drew attention to a tumor that was already there. Similarly, there is no convincing evidence that cell phones, power lines, or everyday environmental exposures cause sarcoma.

Most sarcomas happen by chance, with no clear reason. But researchers have identified several factors that can raise a person's risk.

Inherited Gene Changes (Hereditary Conditions)

Some people are born with gene changes (mutations) that they inherited from a parent. These mutations can make it more likely for certain cancers, including sarcomas, to develop.

  • Li-Fraumeni syndrome: This is caused by a mutation in the TP53 gene. People with this condition have a much higher lifetime risk of several cancers, including soft tissue sarcomas and osteosarcoma. It most commonly affects children and young adults.

  • Neurofibromatosis type 1 (NF1): Also called von Recklinghausen disease, this condition causes benign nerve tumors to grow throughout the body. In some cases, these tumors can turn into a malignant (cancerous) peripheral nerve sheath tumor (MPNST), which is a type of sarcoma.

  • Familial retinoblastoma: People who carry the RB1 gene mutation (which causes retinoblastoma, a childhood eye cancer) have a higher risk of osteosarcoma and other sarcomas later in life.

  • Gardner syndrome: A condition caused by mutations in the APC gene. It is mostly known for causing colon polyps and colon cancer, but it also raises the risk of a type of soft tissue tumor called a desmoid tumor, which grows in the connective tissue of the abdomen. Desmoid tumors are not technically malignant (they do not spread to other organs), but they can grow aggressively and cause serious problems.

  • Werner syndrome: A rare condition that causes premature aging and raises the risk of several cancers, including soft tissue sarcoma.

  • Gorlin syndrome (Basal Cell Nevus Syndrome): Linked to an increased risk of several cancers, including rhabdomyosarcoma.

    • Age: The risk of sarcoma varies by type and age group. Osteosarcoma and Ewing sarcoma are most common in children, teens, and young adults, often during periods of rapid bone growth. Rhabdomyosarcoma is also most common in children. Other sarcomas, like liposarcoma, leiomyosarcoma, and undifferentiated pleomorphic sarcoma, are much more common in adults over age 40 or 50.

    Other Factors

    • Previous Radiation Therapy: Radiation therapy is a common and effective treatment for many types of cancer. However, in a small number of people, radiation can damage the DNA of healthy cells in the treated area, sometimes causing a new cancer called a radiation-induced sarcoma to develop years or even decades later. This is uncommon, but it is a recognized risk. The most common types of radiation-induced sarcoma are osteosarcoma and undifferentiated pleomorphic sarcoma. The benefits of radiation therapy for treating the original cancer far outweigh this small risk.

    • Lymphedema: Lymphedema is a condition where fluid builds up in tissues and causes swelling, often in the arm or leg. It can happen after surgery or radiation that damages lymph vessels. Long-standing lymphedema can very rarely lead to a type of sarcoma called angiosarcoma (a cancer that grows in blood or lymph vessel walls). This specific combination, angiosarcoma arising in the arm after breast cancer treatment, is sometimes called Stewart-Treves syndrome.

    • Chemical Exposures: Exposure to certain industrial chemicals has been linked to a higher risk of soft tissue sarcomas, particularly a rare type called hepatic angiosarcoma (angiosarcoma of the liver). Chemicals associated with this risk include:

      • Vinyl chloride: A chemical used to make PVC plastic.

  • Arsenic: A naturally occurring metal found in some pesticides, industrial processes, and groundwater.

  • Thorotrast: A radioactive contrast dye that was used in medical imaging in the past but is no longer used.

  • Dioxins (toxic chemicals formed as byproducts of industrial processes) have also been studied as a possible risk factor.

  • Kaposi Sarcoma and HIV/AIDS: Kaposi sarcoma is a type of sarcoma that involves the skin, lymph nodes, and internal organs. It is caused by human herpesvirus 8 (HHV-8) and is much more common in people who have weakened immune systems, especially those with HIV/AIDS. With the widespread use of antiretroviral therapy (ART) for HIV, Kaposi sarcoma has become much less common in developed countries.

    • What’s Next: The next page in this guide describes How You Can Lower Your Risk of Sarcoma. If you would like to read another page in this guide, return to the Sarcoma 101 Guides page or choose another topic. 

    Sources:

  • Malkin D. Li-fraumeni syndrome. Genes & Cancer. 2011;2(4):475–484. https://pubmed.ncbi.nlm.nih.gov/21779515/

  • Brady MS, Gaynor JJ, Brennan MF. Radiation-associated sarcoma of bone and soft tissue. Archives of Surgery. 1992;127(12):1379–1385. https://pubmed.ncbi.nlm.nih.gov/1365680/

    • Risk Factors for Sarcoma

    Last updated and reviewed on May 27, 2026.

    A risk factor is anything that increases the chance that a person will develop a certain disease. Having one or more risk factors does not mean you will definitely get sarcoma; many people with sarcoma have no known risk factors at all. And many people with known risk factors never develop the disease. Still, knowing the risk factors can help you and your doctor watch for problems and have informed conversations about your health.

    What does NOT cause sarcoma: It is important to note that injuries and bumps do not cause sarcoma. Sometimes a person notices a sarcoma after an injury, but the injury did not cause the cancer; the injury simply drew attention to a tumor that was already there. Similarly, there is no convincing evidence that cell phones, power lines, or everyday environmental exposures cause sarcoma.

    Most sarcomas happen by chance, with no clear reason. But researchers have identified several factors that can raise a person's risk.

    Inherited Gene Changes (Hereditary Conditions)

    Some people are born with gene changes (mutations) that they inherited from a parent. These mutations can make it more likely for certain cancers, including sarcomas, to develop.

    • Li-Fraumeni syndrome: This is caused by a mutation in the TP53 gene. People with this condition have a much higher lifetime risk of several cancers, including soft tissue sarcomas and osteosarcoma. It most commonly affects children and young adults.

    • Neurofibromatosis type 1 (NF1): Also called von Recklinghausen disease, this condition causes benign nerve tumors to grow throughout the body. In some cases, these tumors can turn into a malignant (cancerous) peripheral nerve sheath tumor (MPNST), which is a type of sarcoma.

    • Familial retinoblastoma: People who carry the RB1 gene mutation (which causes retinoblastoma, a childhood eye cancer) have a higher risk of osteosarcoma and other sarcomas later in life.

    • Gardner syndrome: A condition caused by mutations in the APC gene. It is mostly known for causing colon polyps and colon cancer, but it also raises the risk of a type of soft tissue tumor called a desmoid tumor, which grows in the connective tissue of the abdomen. Desmoid tumors are not technically malignant (they do not spread to other organs), but they can grow aggressively and cause serious problems.

    • Werner syndrome: A rare condition that causes premature aging and raises the risk of several cancers, including soft tissue sarcoma.

    • Gorlin syndrome (Basal Cell Nevus Syndrome): Linked to an increased risk of several cancers, including rhabdomyosarcoma.

    Age: The risk of sarcoma varies by type and age group. Osteosarcoma and Ewing sarcoma are most common in children, teens, and young adults, often during periods of rapid bone growth. Rhabdomyosarcoma is also most common in children. Other sarcomas, like liposarcoma, leiomyosarcoma, and undifferentiated pleomorphic sarcoma, are much more common in adults over age 40 or 50.

    Other Factors

    • Previous Radiation Therapy: Radiation therapy is a common and effective treatment for many types of cancer. However, in a small number of people, radiation can damage the DNA of healthy cells in the treated area, sometimes causing a new cancer called a radiation-induced sarcoma to develop years or even decades later. This is uncommon, but it is a recognized risk. The most common types of radiation-induced sarcoma are osteosarcoma and undifferentiated pleomorphic sarcoma. The benefits of radiation therapy for treating the original cancer far outweigh this small risk.

    • Lymphedema: Lymphedema is a condition where fluid builds up in tissues and causes swelling, often in the arm or leg. It can happen after surgery or radiation that damages lymph vessels. Long-standing lymphedema can very rarely lead to a type of sarcoma called angiosarcoma (a cancer that grows in blood or lymph vessel walls). This specific combination, angiosarcoma arising in the arm after breast cancer treatment, is sometimes called Stewart-Treves syndrome.

    • Chemical Exposures: Exposure to certain industrial chemicals has been linked to a higher risk of soft tissue sarcomas, particularly a rare type called hepatic angiosarcoma (angiosarcoma of the liver). Chemicals associated with this risk include:

      • Vinyl chloride: A chemical used to make PVC plastic.

      • Arsenic: A naturally occurring metal found in some pesticides, industrial processes, and groundwater.

      • Thorotrast: A radioactive contrast dye that was used in medical imaging in the past but is no longer used.

      • Dioxins (toxic chemicals formed as byproducts of industrial processes) have also been studied as a possible risk factor.

      • Kaposi Sarcoma and HIV/AIDS: Kaposi sarcoma is a type of sarcoma that involves the skin, lymph nodes, and internal organs. It is caused by human herpesvirus 8 (HHV-8) and is much more common in people who have weakened immune systems, especially those with HIV/AIDS. With the widespread use of antiretroviral therapy (ART) for HIV, Kaposi sarcoma has become much less common in developed countries.

    What’s Next: The next page in this guide describes How You Can Lower Your Risk of Sarcoma. If you would like to read another page in this guide, return to the Sarcoma 101 Guides page or choose another topic. 

    Sources:

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