Soft tissue sarcoma risk factors

Caused by genetics

Caused by infection

Caused by radiation

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-Tetrachlorodibenzo-para-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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Find out more about the definitions and evidence for this data

Learn how attributable risk is calculated

References

  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 122*. Accessed October 2018.
  3. World Cancer Research Fund / American Institute for Cancer Research. Continuous Update Project Findings & Reports. Accessed October 2018.
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International Agency for Research on Cancer (IARC) classifies the role of this risk factor in cancer development.[1] 100% of Kaposi sarcoma cases in the UK are caused by Kaposi sarcoma herpes virus/human herpesvirus 8 (KSHV/HHV8) infection.[2]

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.[3] 

Kaposi sarcoma is an AIDS-defining malignancy (meaning its occurrence in an HIV-positive person constitutes an AIDS diagnosis), of Kaposi sarcoma risk is 3,600 times higher in HIV-positive people compared with the general population, a meta-analysis showed.[4] 

Leiomyosarcoma risk may also be elevated in people with HIV, though this appears to be causatively linked to Epstein-Barr virus infection.[5-7]

References

  1. International Agency for Research on Cancer. List of Classifications by cancer sites with sufficient or limited evidence in humans, Volumes 1 to 122. Accessed October 2018.
  2. 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.
  3. Mesri EA, Cesarman E, Boshoff C. Kaposi's sarcoma and its associated herpesvirus. Nat Rev Cancer. 2010 Oct;10(10):707-19.
  4. Grulich AE, van Leeuwen MT, Falster MO, et al. Incidence of cancers in people with HIV/AIDS compared with immunosuppressed transplant recipients: a meta-analysis. Lancet 2007;370(9581):59-67.
  5. Bhatia K, Shiels MS, Berg A, et al. Sarcomas other than Kaposi sarcoma occurring in immunodeficiency: interpretations from a systematic literature review. Curr Opin Oncol 2012;24(5):537-46.
  6. Simard EP, Shiels MS, Bhatia K, et al. Long-term cancer risk among people diagnosed with AIDS during childhood. Cancer Epidemiol Biomarkers Prev 2012;21(1):148-54.
  7. International Agency for Research on Cancer. IARC Monographs on the Evaluation of Carcinogenic Risks to Humans. Volume 100B (2012). A Review of Human Carcinogens: Biological Agents. Lyon:IARC;2012.
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Li-Fraumeni syndrome

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.[1] 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]

Retinoblastoma

(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]

Neurofibromatoses (NF)

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).[5] 

Malignant peripheral nerve sheath tumours are associated with NF1.[6]

References

  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.
  5. National Cancer Intelligence Network. Soft Tissue Sarcomas: incidence and survival rates in England. NCIN Data Briefing. London: NCIN; 2011.
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International Agency for Research on Cancer (IARC) classifies the role of this risk factor in cancer development.[1]

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.[2] Typical sources of exposure are radiotherapy, medical diagnostics (e.g. X-rays), and natural background radiation (e.g. radon).[3] Iodine-131 is a radioactive isotope which can be used to treat hyperthyroidism and some types of thyroid cancer.

Radiotherapy during childhood

Childhood cancer survivors who received radiotherapy have around 16 times higher STS risk compared to those who did not, a British cohort study showed.[4] The risk increases with cumulative radiation dose, with those receiving the highest dose at around 50 times increased STS risk.[4] Receiving chemotherapy as well as radiotherapy increases STS risk even further.[4]

Radiotherapy as an adult

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.[2] Cancer survivors who did not receive radiotherapy also have an increased second STS risk, but to a far lesser extent.[2] STS risk is around five-and-a-half times higher in women who have received radiotherapy for breast cancer compared to the general female population, a meta-analysis showed.[5] Breast cancer survivors who did not receive radiotherapy also have an increased STS risk but to a far lesser extent.[5]

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.[2]

See also

See IARC's classifications on the role of this risk factor

Find out more about the definitions and evidence for this data

Learn how attributable risk is calculated

References

  1. International Agency for Research on Cancer. List of Classifications by cancer sites with sufficient or limited evidence in humans, Volumes 1 to 122. Accessed October 2018.
  2. Berrington de Gonzalez A, Kutsenko A, Rajaraman P. Sarcoma risk after radiation exposure. Clin Sarcoma Res 2012;2(1):18.
  3. 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.
  4. 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.
  5. Grantzau T, Overgaard J. Risk of second non-breast cancer among patients treated with and without postoperative radiotherapy for primary breast cancer: A systematic review and meta-analysis of population-based studies including 522,739 patients. Radiotherapy and Oncology 2016;121(3):402-413.
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International Agency for Research on Cancer (IARC) classifies the role of this risk factor in cancer development.[1]

Polychlorophenols

Polychlorophenols are used in pesticides, herbicides and disinfectants. Crop protection product manufacturing workers do not have an increased risk of dying from soft tissue sarcoma (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.

2,3,7,8-Tetrachlorodibenzodioxin (TCDD)

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. STS cancer risk is not significantly different in people with high exposure to TCDD however, mortality risk is 60% higher in people with high exposure to TCDD compared with non-exposed people a meta-analysis showed.[4] However this analysis did not determine if STS was defined by anatomical code (ICD-10 C49) or morphology so these findings should be interpreted with caution.

See also

See IARC's classifications on the role of this risk factor

Find out more about the definitions and evidence for this data

Learn how attributable risk is calculated

See more information on how occupational exposures can be a cause of cancer

References

  1. International Agency for Research on Cancer. List of Classifications by cancer sites with sufficient or limited evidence in humans, Volumes 1 to 122. Accessed October 2018.
  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.
  4. Xu J, Ye Y, Huang F, et al. Association between dioxin and cancer incidence and mortality: a meta-analysis. Scientific Reports 2016;6(1).
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