Soft tissue sarcoma risk factors

Less than 1% of soft tissue sarcoma cases in the UK are preventable.[1]

Soft tissue sarcoma risk is associated with a number of risk factors.[2,3]

Soft Tissue Sarcoma Risk Factors

  Increases risk Decreases risk
'Sufficient' or 'convincing' evidence
  • Human immunodeficiency virus (HIV) type 1[a]
  • Kaposi sarcoma herpes virus[a]
'Limited' or 'probable' evidence
  • Polychlorophenols or their sodium salts (combined exposures)
  • Radioiodines, including iodine- 131 2,3,7,8-
  • Tetrachlorodibenzopara-dioxin
International Agency for Research on Cancer (IARC) classification. World Cancer Research Fund/American Institute for Cancer Research (WCRF/AICR) classification does not include soft tissue sarcomas because they are not generally recognised to have a relationship to food, nutrition, and physical activity.
a Kaposi sarcoma

See also

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  1. Calculated by the Statistical Information Team at Cancer Research UK, 2018. Based on Brown KF, Rumgay H, Dunlop C, et al. The fraction of cancer attributable to known risk factors in England, Wales, Scotland, Northern Ireland, and the UK overall in 2015. British Journal of Cancer 2018. and National Cancer Intelligence Network (NCIN) Bone and Soft Tissue Sarcomas UK Incidence and Survival: 1996 to 2010. 2013.
  2. International Agency for Research on Cancer. List of Classifications by cancer sites with sufficient or limited evidence in humans, Volumes 1 to 116*. Accessed October 2016.
  3. World Cancer Research Fund / American Institute for Cancer Research. Continuous Update Project Findings & Reports. Accessed October 2016.
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The International Agency for Research on Cancer (IARC) classifies Kaposi sarcoma herpes virus/human herpesvirus 8 (KSHV/HHV8) and Human immunodeficiency virus type 1 (HIV) as causes of Kaposi sarcoma. Kaposi sarcoma only develops in people with KSHV/HHV8 infection, but most people with KSHV/HHV8 never develop Kaposi sarcoma, and in most Kaposi sarcoma cases, HIV infection is also present.[1] Kaposi sarcoma is an AIDS-defining malignancy (meaning its occurrence in an HIV-positive person constitutes an AIDS diagnosis), and the risk of Kaposi sarcoma in HIV-positive people is more than 3,600 times greater than in the general population, a meta-analysis showed.[2] 100% of Kaposi sarcoma cases in the UK are caused by KHSV/HH8V infection.[3]

Leiomyosarcoma risk may also be elevated in people with HIV, though this appears to be causatively linked to Epstein-Barr virus infection.[4-6]

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Patients receiving immunosuppressant medication after an organ transplant are at around 200 times greater risk of Kaposi sarcoma compared with the general population, a meta-analysis showed.[1]

Children with allergies may have a 40% lower risk of rhabdomyosarcoma, a case-control study showed.[2]

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Exposure to ionising radiation increases soft tissue sarcoma (STS) risk, and risk appears to increase in line with exposure levels, though most evidence on absorbed doses between 5 and 20 Gray (Gy), and many studies define STS by anatomical site rather than morphology.[1] Typical sources of exposure are radiotherapy, medical diagnostics (e.g. X-rays), and natural background radiation (e.g. radon).[2]

Radiotherapy for cancer during childhood appears to have the greatest impact on STS risk, though the magnitude of the increase varies widely between studies, probably related to whether STS are defined by anatomical site or tumour morphology.[1] Childhood cancer survivors who received radiotherapy have around 16 times higher STS risk compared to those who did not, and the risk increases with cumulative radiation dose, with those receiving the highest dose at around 50 times increased STS risk, a British cohort study showed.[3] Receiving chemotherapy as well as radiotherapy increases subsequent STS risk even further.[3]

Radiotherapy for cancer (other than bone sarcoma or STS) during adulthood is associated with a 77% increased risk of second STS compared to the general population, analysis of US cancer registry data shows.[1] Cancer survivors who did not receive radiotherapy also have an increased second STS risk, but to a far lesser extent.[1] STS risk increases with longer time since diagnosis of the first cancer (22% at 1-4 years, 120% at 15+ years), and younger age at diagnosis of the first cancer (83% at age 60-79, 432% at age 20-39); there is also some evidence that risk varies by site of the primary cancer.[1]

Evidence for an increased risk at lower exposure levels comes from a cohort study of atomic bomb survivors with 56 years follow-up, which found risk of STS around doubled per 1Gy exposure.[4] Studies of radiotherapy at doses lower than 5Gy have generally found no increased STS risk, but low sample size preclude firm conclusions.[1]

The International Agency for Research on Cancer (IARC) classifies radioiodines, including Iodine-131, as possible causes of STS, based on limited evidence.[5] Iodine-131 is a radioactive isotope which can be used to treat hyperthyroidism and some types of thyroid cancer.


  1. Berrington de Gonzalez A, Kutsenko A, Rajaraman P. Sarcoma risk after radiation exposure. Clin Sarcoma Res 2012;2(1):18.
  2. Parkin DM, Darby SC. 12. Cancers in 2010 attributable to ionising radiation exposure in the UK. Br J Cancer 2011;105 Suppl 2:S57-65.
  3. Jenkinson HC, Winter DL, Marsden HB, et al. A study of soft tissue sarcomas after childhood cancer in Britain. Br J Cancer 2007;97(5):695-9.
  4. Samartzis D, Nishi N, Cologne J, et al. Ionizing radiation exposure and the development of soft-tissue sarcomas in atomic-bomb survivors. J Bone Joint Surg Am 2013;95(3):222-9.
  5. International Agency for Research on Cancer. List of Classifications by cancer sites with sufficient or limited evidence in humans, Volumes 1 to 105*. Available from: Accessed May 2013.
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Sarcoma (of soft tissue or bone) in people who have had previous cancer represents 7.5% of all sarcoma cases, a cohort study showed.[1] Around a quarter of these second sarcomas are thought to be related to radiotherapy for the primary tumour.[1] The most frequent histological subtypes of second sarcoma were malignant fibrous histiocytoma/myxofibrosarcoma, gastrointestinal stromal tumour, and leiomyosarcoma; and the most frequent primary tumours in people with second sarcomas were breast, urological, skin and gynaecological.[1] In breast cancer survivors, sarcoma risk is often (but not always) associated with lymphoedema.

The incidence of second sarcoma has increased more rapidly than that of primary sarcoma, probably because more intensive cancer treatments mean more people survive their primary cancer, but that treatment is also more likely to cause second cancer.[1]

People who survived cancer in childhood have eight times the general population risk of soft tissue sarcoma (STS) five years or more after their primary diagnosis, a large cohort study showed.[2] The risk is higher for those who had their primary cancer at a younger age, who received radiotherapy, and/or whose primary cancer was a central nervous system tumour, Hodgkin lymphoma, Wilms tumour, or soft tissue or bone sarcoma.[2]


  1. Bjerkehagen B, Småstuen MC, Hall KS, et al. Incidence and mortality of second sarcomas - A population-based study. Eur J Cancer 2013; doi: 10.1016/j.ejca.2013.05.017.
  2. Friedman DL, Whitton J, Leisenring W, et al. Subsequent neoplasms in 5-year survivors of childhood cancer: the Childhood Cancer Survivor Study. J Natl Cancer Inst 2010;102(14):1083-95.
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Neurofibromatoses (NF) Open a glossary item soft tissue sarcoma (STS) risk is at least 122 times higher in people with NF type 1 versus the general population (this figure is for ICD-10 codes C47 and C49, which capture less than half of all STS).[1] Malignant peripheral nerve sheath tumours are associated with NF1.[2]

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Li-Fraumeni syndrome Open a glossary item soft tissue sarcoma (STS) and osteosarcoma risk among people aged under 20 may be up to 500 times higher in TP53 mutation (the usual cause of Li-Fraumeni syndrome) carriers versus the general population. STS tends to occur at a younger age in TP53 mutation carriers than in the general population,[1] Rhabdomyosarcoma, leiomyosarcoma, liposarcoma and histiosarcoma are associated with TP53 mutation.[1]

Genetic conditions: Hereditary retinoblastoma

Hereditary retinoblastoma soft tissue sarcoma (STS) risk is 124-145 times higher in hereditary retinoblastoma survivors compared with the general population, a cohort study showed.[2] This is probably due to a combination of genetic susceptibility and radiotherapy for the primary cancer.[2,3] Leiomyosarcoma, fibrosarcoma and rhabdomyosarcoma are associated with hereditary retinoblastoma.[4]


  1. Schneider K, Garber J. Li-Fraumeni Syndrome. In: Pagon RA, Bird TD, Dolan CR, et al, editor. GeneReviews™ [internet]. Seattle (WA): University of Washington, 1999 [Updated 2010].
  2. Wong JR, Morton LM, Tucker MA, et al. Risk of subsequent malignant neoplasms in long-term hereditary retinoblastoma survivors after chemotherapy and radiotherapy.. J Clin Oncol 2014;32(29):3284-90.
  3. Ognjanovic S, Olivier M, Bergemann TL, et al. Sarcomas in TP53 germline mutation carriers: a review of the IARC TP53 database. Cancer 2012;118(5):1387-96.
  4. Kleinerman RA, Schonfeld SJ, Tucker MA. Sarcomas in hereditary retinoblastoma. Clin Sarcoma Res 2012;2(1):15.
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Polychlorophenols is classified by The International Agency for Research on Cancer (IARC) as a possible cause of soft tissue sarcoma (STS), based on limited evidence.[1]

Polychlorophenols are used in pesticides, herbicides and disinfectants. Crop protection product manufacturing workers do not have an increased risk of dying from STS, a meta-analysis showed.[2] Similarly, men using pesticides on a commercial basis in the UK have no increased risk of being diagnosed with or dying from STS, a large cohort study showed.[3] However, both these studies defined STS by anatomical site code rather than morphology, so their findings should be interpreted with caution.


  1. International Agency for Research on Cancer. List of Classifications by cancer sites with sufficient or limited evidence in humans, Volumes 1 to 105*. Available from: Accessed May 2013.
  2. Jones DR, Sutton AJ, Abrams KR, et al. Systematic review and meta-analysis of mortality in crop protection product manufacturing workers. Occup Environ Med 2009;66(1):7-15.
  3. Frost G, Brown T, Harding AH. Mortality and cancer incidence among British agricultural pesticide users. Occup Med (Lond) 2011;61(5):303-10.
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2,3,7,8-Tetrachlorodibenzodioxin (TCDD) is classified by the International Agency for Research on Cancer (IARC) as a possible cause of soft tissue sarcoma (STS), based on limited evidence.[1]

TCDD are by-products of industrial processes and combustion, and industries with possible exposure include metal and waste recycling, agriculture/horticulture, pesticide manufacture, and pulp manufacture. It is estimated that fewer than 30 STS cases in Great Britain in 2004 were attributable to TCDD exposure;[2] this may be an underestimate as STS was defined by anatomical site (ICD-10 C49) rather than morphology in this study.


  1. International Agency for Research on Cancer. List of Classifications by cancer sites with sufficient or limited evidence in humans, Volumes 1 to 105*. Available from: Accessed May 2013.
  2. Brown T, Young C, Rushton L; British Occupational Cancer Burden Study Group.Occupational cancer in Britain. Remaining cancer sites: brain, bone, soft tissue sarcoma and thyroid. Br J Cancer 2012;107 Suppl 1:S85-91.
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Children who are heavier at birth have a higher risk of rhabdomyosarcoma, with an 18% increase per 500g increase in birth weight, a case-control study found.[1]

Women whose body mass index (BMI) is 30 or more have a 74% higher risk of endometrial stromal sarcoma compared with women whose BMI is 25 or less, a pooled analysis showed.[2]

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Stewart-Treves syndrome is a rare condition in which angiosarcoma develops at the site of chronic lymphoedema Open a glossary item (swelling of body tissues due to impaired lymphatic drainage).[1] It is thought most lymphoedema cases occur in the arm following breast cancer treatment, though cases an occur in the leg following pelvic or genital cancer treatment, and there are also congenital and infectious aetiologies.[2] Up to one in five breast cancer survivors who undergo axillary clearance develop lymphoedema, a meta analysis showed.[3]


  1. Young RJ, Brown NJ, Reed MW, et al. Angiosarcoma. Lancet Oncol 2010;11(10):983-91.
  2. Rockson SG. Lymphedema. Am J Med 2001;110(4):288-95.
  3. DiSipio T, Rye S, Newman B, et al. Incidence of unilateral arm lymphoedema after breast cancer: a systematic review and meta-analysis. Lancet Oncol 2013;14(6):500-15.
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Children who have a congenital umbilical or inguinal hernia have around three times the risk of Ewing sarcoma of the soft tissue or bone, a meta-analysis showed.[1] Soft tissue sarcoma (STS) risk is higher in children with congenital disorders of the nervous system, a cohort study showed.[2]

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Among HIV-positive men who have never used highly active antiretroviral therapy (HAART), Kaposi sarcoma risk appears to be 43% lower in current smokers compared with non-current smokers, a cohort study showed.[1] But the relationship between smoking and other soft tissue sarcoma (STS) subtypes, in non HIV-positive populations, remains unclear.[2]

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The World Cancer Research Fund/American Institute for Cancer Research (WCRF/AICR) concluded that any study investigating possible links between soft tissue sarcoma (STS) and food, nutrition and physical activity would be unlikely to be fruitful because these cancers are diverse and rare, and therefore further investigation is unlikely to be warranted.[1]

Women with a history of diabetes have more than double the risk of endometrial stromal sarcoma (a type of uterine sarcoma) compared with non-diabetic women, a pooled analysis shows;[2] however, treatment for diabetes may impact on cancer risk but was not factored into this analysis.[3] Associations between other STS subtypes and diabetes remain unclear.


  1. World Cancer Research Fund/American Institute for Cancer Research. Food, Nutrition, Physical Activity, and the Prevention of Cancer: a Global Perspective. Washington DC: AICR; 2007.
  2. Ognjanovic S, Carozza SE, Chow EJ, et al. Birth characteristics and the risk of childhood rhabdomyosarcoma based on histological subtype. Br J Cancer 2010;102(1):227-31.
  3. Thakkar B, Aronis KN, Vamvini MT, et al. Metformin and Sulfonylureas in Relation to Cancer Risk in Type II Diabetes Patients: A Meta-analysis using primary data of published studies. Metabolism 2013;62(7):922-34.
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