Risk factors for soft tissue sarcoma

A risk factor is something that increases the risk of developing cancer. It could be a behaviour, substance or condition. Most cancers are the result of many risk factors. But sometimes soft tissue sarcoma develops in people who don’t have any of the risk factors described below.

Soft tissue sarcoma can develop at any age, but it occurs most often in people older than 50 years of age.

Risk factors are generally listed in order from most to least important. But in most cases, it is impossible to rank them with absolute certainty.

Risk factors

Radiation therapy

Genetic conditions

Chronic lymphedema

Viral infections

Vinyl chloride

Thorium dioxide

There is convincing evidence that the following factors increase your risk for soft tissue sarcoma.

Radiation therapy

People who receive radiation therapy as treatment for another cancer have a higher risk of developing soft tissue sarcoma. This is particularly true for people who received radiation therapy for Hodgkin lymphoma, non-Hodgkin lymphoma or breast cancer. Soft tissue sarcoma often develops in the area of the body that was treated with radiation. The average time between coming into contact with radiation and a diagnosis of soft tissue sarcoma is 10 years.

Less than 5% of sarcomas are caused by radiation therapy. The benefit of using radiation therapy to treat cancer outweighs the increased risk of developing soft tissue sarcoma.

Genetic conditions

The following rare inherited conditions increase the risk of developing soft tissue sarcoma.

Retinoblastoma is a cancerous tumour that develops in the retina (the membrane at the back of the eye). It occurs mostly in children.

Werner syndrome causes people to age very rapidly after puberty.

Li-Fraumeni syndrome is associated with an increased risk of developing several types of cancer, including soft tissue sarcoma.

Neurofibromatosis type 1 (also known as von Recklinghausen disease) affects the nervous system. It is a condition that causes tumours to form in nerve tissues in and under the skin. People with neurofibromatosis type 1 have a higher risk of developing certain cancers, including soft tissue sarcoma.

Gardner syndrome causes large numbers of polyps in the colon, along with tumours in the bone and soft tissues.

Chronic lymphedema

Lymphedema is a buildup of fluid in body tissues. Long-term, or chronic, lymphedema can cause lymphangiosarcoma (also called Stewart-Treves syndrome). This is a rare type of soft tissue sarcoma that develops in lymph vessels

Viral infections

The following viral infections increase your risk of developing soft tissue sarcoma.

Kaposi sarcoma herpes virus (KSHV) is also called human herpes virus 8 (HHV-8) or Kaposi sarcoma-associated herpes. This virus can cause a rare type of sarcoma called Kaposi sarcoma (KS).

Human immunodeficiency virus (HIV) increases the risk of developing KS and soft tissue sarcoma.

Vinyl chloride

Vinyl chloride is used in the plastics industry to make polyvinyl chloride (PVC), which is used in many products. Coming into contact with vinyl chloride at work increases the risk of developing angiosarcoma of the liver, a type of soft tissue sarcoma.

Thorium dioxide

Thorium dioxide (Thorotrast) is a radioactive solution that was used as a contrast medium in the 1950s to improve x-ray images of the blood vessels and arteries, nasal passages and sinus cavities. Thorium dioxide was injected into a vein and it collected in the liver, spleen and bone marrow. People who were exposed to thorium dioxide have a greater risk of developing angiosarcoma of the liver, which is a type of soft tissue sarcoma. Higher doses of thorium dioxide increase the risk. The cancer may develop as many as 45 years after exposure.

Possible risk factors

The following factors have been linked with soft tissue sarcoma, but there is not enough evidence to show for sure that they are risk factors. More research is needed to clarify the role of these factors for soft tissue sarcoma.

  • previous chemotherapy treatment
  • personal history of cancer
  • coming into contact with dioxin at work

Questions to ask your healthcare team

To make the decisions that are right for you, ask your healthcare team questions about risks.

Expert review and references

  • American Cancer Society. Risk Factors for Soft Tissue Sarcomas. 2018: https://www.cancer.org/cancer/soft-tissue-sarcoma/causes-risks-prevention/risk-factors.html.
  • American Society of Clinical Oncology. Sarcoma, Soft Tissue: Risk Factors. 2017: https://www.cancer.net/cancer-types/sarcoma-soft-tissue/risk-factors.
  • Baan R, Grosse Y, Straif K, et al . A review of human carcinogens: Part F: chemical agents and related occupations. Lancet Oncology. Elsevier; 2009.
  • Berwick M, Wiggins C . Soft tissue sarcoma. Thun MJ (ed.). Schottenfeld and Fraumeni Cancer Epidemiology and Prevention. 4th ed. New York, NY: Oxford University Press; 2018: 43:829-838.
  • Bouvard V, Baan R, Straif K, et al . A review of human carcinogens: Part B: biological agents. Lancet Oncology. Elsevier; 2009.
  • Briggs NC, Levine RS, Hall HI, et al . Occupational risk factors for selected cancers among African American and White men in the United States. American Journal of Public Health. Washington, DC: American Public Health Association; 2003.
  • Collins JJ, Bodnar KM, Aylward LL, et al . Mortality risk among workers with exposure to dioxins. Occupational Medicine. 2016: 66(9):706-712. https://academic.oup.com/occmed/article/66/9/706/2649205.
  • Collins JJ, Bodner K, Aylward LL, Wilken M, Bodnar CM . Mortality rates among trichlorophenol workers with exposure to 2,3,7,8-tetrachlorodibenzo-p-dioxin. American Journal of Epidemiology. Oxford Journals; 2009.
  • Darragh CT, Wheless LE, Stebbins WG, et al. . Unusual cutaneous malignancies. Raghavan D, et al (eds.). Textbook of Uncommon Cancer. 5th ed. Wiley Blackwell; 2017: 69:976-993.
  • Engels EA, Pfeiffer RM, Fraumeni JF Jr, Kasiske BL, Israni AK, Snyder JJ et al . Spectrum of cancer risk among US solid organ transplant recipients. JAMA. 2011.
  • Gennaro V, Ceppi M, Crosignani P, Montanaro F . Reanalysis of updated mortality among vinyl and polyvinyl chloride workers: Confirmation of historical evidence and new findings. BMC Public Health. Biomed Central; 2008.
  • Henderson TO, Whitton J, Stovall M, et al . Secondary sarcomas in childhood cancer survivors: a report from the Childhood Cancer Survivor Study. Journal of the National Cancer Institute. Oxford University Press; 2007.
  • Hoppin JA, Tolbert PE, Herrick RF, et al . Occupational chlorophenol exposure and soft tissue sarcoma risk among men aged 30-60 years. American Journal of Epidemiology. Oxford Journals; 1998.
  • International Agency for Research on Cancer (IARC). Volume 78: Ionizing radiation part 2: some internally deposited radionuclides. 2001: http://monographs.iarc.fr/ENG/Monographs/vol78/mono78.pdf.
  • International Agency for Research on Cancer (IARC). Volume 75: Ionizing Radiation Part 1: X- and Gamma (y)-Radiation, and Neutrons. 2000: http://monographs.iarc.fr/ENG/Monographs/vol75/mono75.pdf.
  • International Agency for Research on Cancer (IARC). Volume 70: Epstein-Barr virus and Kaposi's sarcoma herpesvirus/human herpesvirus 8. 1997: http://monographs.iarc.fr/ENG/Monographs/vol70/mono70.pdf.
  • International Agency for Research on Cancer (IARC). Volume 97: 1,3-Butadiene, ethylene oxide and vinyl halides (vinyl fluoride, vinyl chloride and vinyl bromide). 2008: http://monographs.iarc.fr/ENG/Monographs/vol97/mono97.pdf.
  • International Agency for Research on Cancer (IARC). Volume 69: Polychlorinated dibenzo-para-dioxins and polychlorinated dibenzofurans. 1997: http://monographs.iarc.fr/ENG/Monographs/vol69/mono69.pdf.
  • International Agency for Research on Cancer (IARC). Volume 100D: Radiation: a review of human carcinogens. 2011: http://monographs.iarc.fr/ENG/Monographs/vol100D/mono100D.pdf.
  • International Agency for Research on Cancer (IARC). Volume 100F: Chemical agents and related occupations: a review of human carcinogens. 2012: http://monographs.iarc.fr/ENG/Monographs/vol100F/index.php.
  • International Agency for Research on Cancer (IARC). Volume 100B: Biological agents: a review of human carcinogens. 2012: http://monographs.iarc.fr/ENG/Monographs/vol100B/mono100B.pdf.
  • Ishida Y, Maeda M, Adachi S, et al . Secondary bone/soft tissue sarcoma in childhood cancer survivors: a nationwide hospital-based case-series study in Japan . Japanese Journal of Clinical Oncology . 2018 : 48(9):801–814 . https://academic.oup.com/jjco/article/48/9/806/5058984.
  • Jenkinson HC, Winter DL, Marsden HB, et al . A study of soft tissue sarcomas after childhood cancer in Britain. British Journal of Cancer (BJC). Nature Publishing Group; 2007.
  • Kempf W, Mertz KD, Hofbauer GFL, Tinguely M . Skin cancer in organ transplant recipients. Pathobiology. 2013.
  • Mundt KA, Dell LD, Crawford L, Gallagher AE . Quantitative estimated exposure to vinyl chloride and risk of angiosarcoma of the liver and hepatocellular cancer in the US industry-wide vinyl chloride cohort: mortality update through 2013. Occupational and Environmental Medicine. 2017: 74(10):709-716. https://oem.bmj.com/content/oemed/74/10/709.full.pdf.
  • National Cancer Institute. Adult Soft Tissue SarcomaTreatment (PDQ®) Health Professional Version. 2018: https://www.cancer.gov/types/soft-tissue-sarcoma/hp/adult-soft-tissue-treatment-pdq.
  • National Toxicology Program. 14th Report on Carcinogens. Department of Health and Human Services; 2016: http://ntp.niehs.nih.gov/pubhealth/roc/index-1.html.
  • Neglia JP, Friedman DL, Yasui Y, Mertens AC, Hammond S, Stovall M, Donaldson SS, Meadows AT, Robison LL . Second malignant neoplasms in five-year survivors of childhood cancer: childhood cancer survivor study. Journal of the National Cancer Institute. Oxford University Press; 2001.
  • O'Brien MM, Donaldson SS, Balise RR, et al . Second malignant neoplasms in survivors of pediatric Hodgkin's lymphoma treated with low-dose radiation and chemotherapy. Journal of Clinical Oncology. American Society of Clinical Oncology; 2010.
  • Pesatori AC, Consonni D, Rubagotti M, Grillo P, Bertazzi PA . Cancer incidence in the population exposed to dioxin after the "Seveso accident": twenty years of follow-up. Environmental Health. BioMed Central; 2009.
  • Peters S, Olencki T . Unusual cutaneous malignancies. Raghavan D, Blanke CD, Honson DH, et al (eds.). Textbook of Uncommon Cancer. 4th ed. Wiley Blackwell; 2012: 49:667-684.
  • Rihana N, Nanjappa S, Sullivan C, et al . Malignancy trends in HIV-infected patients over the past 10 years in a single-center retrospective observational study in the United States. Cancer Control: Journal of the Moffitt Cancer Center. 2018: 25(1):1073274818797955. https://journals.sagepub.com/doi/pdf/10.1177/1073274818797955.
  • Salminen SH, Sampo MM, Bohling TO, et al . Radiation-associated sarcoma after breast cancer in a nationwide population: Increasing risk of angiosarcoma. Cancer Medicine. 2018: 7(9):4825-4835. http://onlinelibrary.wiley.com/journal/10.1002/(ISSN)2045-7634.
  • Schwartz RA, & Fernandez G . Stewart-Treves syndrome. eMedicine.com. Omaha: eMedicine, Inc; 2009.
  • Silverberg MJ, Chao C, Leyden WA, et al . HIV infection, immunodeficiency, viral replication, and the risk of cancer. Cancer Epidemiology, Biomarkers & Prevention. American Association for Cancer Research; 2011.
  • Singer S, Tap WD, Crago AM, O'Sullivan, B . Soft tissue sarcoma. DeVita VT Jr, Lawrence TS, & Rosenberg SA. Cancer: Principles and Practice of Oncology. 10th ed. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins; 2015: 90:1253-1291.
  • Toro JR, Travis LB, Wu HJ, et al . Incidence patterns of soft tissue sarcomas, regardless of primary site, in the surveillance, epidemiology and end results program, 1978-2001: An analysis of 26,758 cases. International Journal of Cancer. Hoboken, NJ: Wiley-Liss, Inc; 2006.
  • Zambon P, Ricci P, Bovo E, Casula A, Gattolin M, Fiore AR, Chiosi F, Guzzinati S . Sarcoma risk and dioxin emissions from incinerators and industrial plants: a population-based case-control study (Italy). Environmental Health. BioMed Central; 2007.

Reducing your risk for soft tissue sarcoma

You can reduce your risk of soft tissue sarcoma by reducing your exposure to certain chemicals. Learn about reducing your risk of soft tissue sarcoma.