Bone sarcoma risk

The estimated lifetime risk of being diagnosed with bone sarcoma is 1 in 1600 (less than 1%) for females, and 1 in 1600 (less than 1%) for males born in 1961 in the UK. [1]

These figures have been calculated on the assumption that the possibility of having more than one diagnosis of bone sarcoma over the course of a lifetime is very low ('Current Probability' method).[2]

See also

Lifetime risk for all cancers combined and cancers compared

Bone sarcoma incidence statistics

How risk is calculated

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References

  1. Lifetime risk estimates calculated by the Cancer Intelligence Team at Cancer Research UK 2023.
  2. Estève J, Benhamou E, Raymond L. Statistical methods in cancer research. Volume IV. Descriptive epidemiology. IARC Sci Publ. 1994;(128):1-302.

About this data

Data is for UK, past and projected cancer incidence and mortality and all-cause mortality rates for those born in 1961, ICD-10 C15.

Calculated by the Cancer Intelligence Team at Cancer Research UK, 2023 (as yet unpublished). Lifetime risk of being diagnosed with cancer for people in the UK born in 1961. Based on method from Esteve et al. 1994 [2], using projected cancer incidence (using data up to 2018) calculated by the Cancer Intelligence Team at Cancer Research UK and projected all-cause mortality (using data up to 2020, with adjustment for COVID impact) calculated by Office for National Statistics. Differences from previous analyses are attributable mainly toslowing pace of improvement in life expectancy, and also to slowing/stabilising increases in cancer incidence.

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Bone sarcoma risk is associated with a number of risk factors.[1,2]

Bone Sarcoma Risk Factors

  Increases risk Decreases risk
'Sufficient' or 'convincing' evidence
  • Plutonium
  • Radium-224 and its decay products
  • Radium-226 and its decay products
  • Radium-228 and its decay products
  • X-radiation, gamma-radiation
  
'Limited' or 'probable' evidence
  • Radioiodines, including Iodine-131
  

International Agency for Research on Cancer (IARC) classification. World Cancer Research Fund/American Institute for Cancer Research (WCRF/AICR) classification does not include bone sarcoma because it is not generally recognised to have a relationship to food, nutrition, and physical activity.

See also

Want to generate bespoke preventable cancers stats statements? Download our interactive statement generator.

Find out more about the definitions and evidence for this data

Learn how attributable risk is calculated

References

  2. World Cancer Research Fund / American Institute for Cancer Research. Continuous Update Project Findings & Reports. Accessed September 2017.
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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 bone sarcoma risk, and risk appears to increase in line with exposure levels, though most evidence is on absorbed doses between 5 and 20 Gray (Gy), and many studies define bone sarcoma 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]

Radiotherapy for cancer during childhood appears to have the greatest impact on bone sarcoma risk; however confounding is possible because much evidence includes populations with primary retinoblastoma, which in itself is associated with bone sarcoma risk.[2,4] Childhood cancer survivors who received around 20Gy from radiotherapy have around 6-38 times higher bone sarcoma risk compared with those who had no radiotherapy or very low doses; bone sarcoma risk increases with radiation doses received.[2,4] Abdominal/pelvic radiotherapy during childhood is associated with 3.1 times increased bone tumour risk, compared with no radiotherapy; radiotherapy to other body sites showed no significant effect in a British cohort study.[5]

Radiotherapy for cancer (other than bone cancer) during adulthood is associated with 2.4 times increased risk of subsequent bone sarcoma compared to the general population analysis of US cancer registry data shows; with higher risk for those diagnosed at younger adult ages.[2] The risk of osteosarcoma is increased by 5.1 times following radiotherapy, but chondrosarcoma risk is not significantly elevated.[6] Bone sarcoma risk in patients with prior adult radiotherapy increases with longer time since diagnosis of the first cancer, and younger age at radiotherapy; there is also some evidence the risk varies by site of the primary cancer (hence the site of the radiotherapy).[2]

Atomic bomb survivors (who have lower overall levels of exposure than patients receiving radiotherapy) have around 7.5 times increased bone sarcoma risk per 1Gy exposure, a cohort study showed.[7] Studies of radiotherapy at doses lower than 5Gy have generally found no increased bone sarcoma risk, but low sample sizes preclude firm conclusions.[2]

IARC classifies radioiodines, including Iodine-131, as possible causes of bone tumours, based on limited evidence.[1] Iodine-131 is a radioactive isotope which can be used to treat hyperthyroidism and some types of thyroid cancer.

Exposure to computed tomography (CT) scans during childhood or adolescence is not associated with an increased risk of bone tumours, a large cohort study showed.[8]

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 August 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. Cancers in 2010 attributable to ionising radiation exposure in the UK. Br J Cancer 2011;105 Suppl 2:S57-65.
  4. Reulen RC, Frobisher C, Winter DL, et al. Long-term risks of subsequent primary neoplasms among survivors of childhood cancer. JAMA. 2011 Jun 8;305(22):2311-9.
  5. Wu LC, Kleinerman RA, Curtis RE, Savage SA, de González AB. Patterns of bone sarcomas as a second malignancy in relation to radiotherapy in adulthood and histologic type. Cancer Epidemiol Biomarkers Prev. 2012 Nov;21(11):1993-9.
  6. Samartzis D, Nishi N, Hayashi M, et al. Exposure to ionizing radiation and development of bone sarcoma: new insights based on atomic-bomb survivors of Hiroshima and Nagasaki. J Bone Joint Surg Am. 2011 Jun 1;93(11):1008-15.
  7. Mathews JD, Forsythe AV, Brady Z, et al. Cancer risk in 680,000 people exposed to computed tomography scans in childhood or adolescence: data linkage study of 11 million Australians. BMJ. 2013 May 21;346:f2360
  8. Schwartz B, Benadjaoud MA, Cléro E, et al. Risk of second bone sarcoma following childhood cancer: role of radiation therapy treatment. Radiat Environ Biophys. 2014 Jan 14.
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People with a parental or sibling history of bone cancer still have only a small risk of developing the disease themselves; less than 1% of bone cancer cases are in people with a first-degree relative with the disease, a large cohort study showed.[1] However, children whose sibling had Ewing sarcoma may have a 16.5 times increased risk of being diagnoses with the same disease; though this figure is based on very few patients so should be interpreted cautiously.[2]

Parent/sibling history of some other cancers is associated with increased bone cancer risk, particularly early-onset bone cancers, further evidence from the same cohort shows.[2] Osteosarcoma risk overall is around doubled in people whose mother or father had rectal cancer or whose father had colon cancer, and increased by 3.6 times in those whose mother had endocrine gland cancer; in people aged under 25 osteosarcoma risk is threefold higher in people whose mother had melanoma, and 1.7 times higher in those whose mother had breast cancer; and in children osteosarcoma risk is around quadrupled when a parent had liver cancer, and doubled with the mother had breast cancer - with around a fivefold increase if the mother was under 45 at her diagnosis.[2] Ewing sarcoma risk too is linked with family history of kidney cancer, with risk 5.6 times higher in children of an affected parent.[2]

References

  1. Hemminki K, Sundquist J, Bermejo JL. How common is familial cancer? Ann Oncol. 2008 Jan;19(1):163-7.
  2. Ji J, Hemminki K. Familial risk for histology-specific bone cancers: an updated study in Sweden. Eur J Cancer. 2006 Sep;42(14):2343-9.
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Genetic predisposition syndromes are associated with a small percentage of bone tumours, typically osteosarcomas.[1,2]

Li-Fraumeni syndrome is a very rare genetic condition associated with increased risk of osteosarcoma and other cancer types.[3] It is usually caused by mutation in the tumour suppressor gene TP53, which is thought to affect 1 in 5,000-20,000 births in the general population.[4] People with TP53 mutation have around 100 times the risk of bone tumours compared with the general population, and bone sarcomas are the most common tumour group in mutation carriers aged 11-20 years.[3]

Heritable (hereditary) retinoblastoma a rare eye cancer diagnosed in children, is caused by a mutation in the tumour suppressor gene RB1.[5] Heritable retinoblastoma survivors have around 200 times increased risk of subsequent bone sarcoma, and more than 400 times increased risk of oesteosarcoma specifically, a British cohort study showed.[6] The increased risk of bone sarcomas in this population is thought to be due to a combination of genetic susceptibility and radiotherapy for the primary cancer.[5,6]

Hereditary multiple exostoses (also known as multiple osteochondromatosis or diaphyseal aclasis) is a rare (affecting up to 1 in 50,000 people) inherited musculoskeletal condition which causes short stature and deformity.[7] In around 5% of people with this condition, benign bone lesions (osteochondromas) transform to chondrosarcomas, a cohort study showed.[8]

Neurofibromatoses (NF) are a group of genetic conditions in which benign (non-invasive) growths affect the nervous system. NF type 1 (NF1) is thought to affect around 1 in 2,700 live births in the UK, whilst NF type 2 (NF2) affects around 1 in 33,000.[9] There is some indication that people with NF1 have an increased risk of bone sarcoma.[10,11]

The RecQ syndromes (Rothmund-Thomson, RAPADILINO, Werner and Bloom syndromes, and Diamond blackfan anaemia) are associated with osteosarcoma predisposition; these rare syndromes also cause growth retardation and dermatological changes.[2]

Osteosarcoma risk is higher in children and adolescents with Down's syndrome, a cohort study showed.[12]

References

  1. Burningham Z, Hashibe M, Spector L, Schiffman JD. The epidemiology of sarcoma. Clin Sarcoma Res 2012;2(1):14.
  2. Calvert GT, Randall RL, Jones KB, et al. At-risk populations for osteosarcoma: the syndromes and beyond. Sarcoma. 2012;2012:152382.
  3. 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].
  4. Ognjanovic S, Olivier M, Bergemann TL, Hainaut P. Sarcomas in TP53 germline mutation carriers: a review of the IARC TP53 database. Cancer 2012;118(5):1387-96.
  5. Kleinerman RA, Schonfeld SJ, Tucker MA. Sarcomas in hereditary retinoblastoma. Clin Sarcoma Res 2012;2(1):15.
  6. MacCarthy A, Bayne AM, Brownbill PA, et al. Second and subsequent tumours among 1927 retinoblastoma patients diagnosed in Britain 1951-2004. Br J Cancer. 2013 Jun 25;108(12):2455-63.
  7. Porter DE, Lonie L, Fraser M, et al. Severity of disease and risk of malignant change in hereditary multiple exostoses. A genotype-phenotype study. J Bone Joint Surg Br. 2004 Sep;86(7):1041-6.
  8. Pedrini E, Jennes I, Tremosini M, et al. Genotype-phenotype correlation study in 529 patients with multiple hereditary exostoses: identification of "protective" and "risk" factors. J Bone Joint Surg Am. 2011 Dec 21;93(24):2294-302.
  9. Evans DG, Howard E, Giblin C, et al. Birth incidence and prevalence of tumor-prone syndromes: Estimates from a UK family genetic register service. Am J Med Genet A 2010; 152A(2):327-32.
  10. Afşar CU, Kara IO, Kozat BK, et al. Neurofibromatosis type 1, gastrointestinal stromal tumor, leiomyosarcoma and osteosarcoma: four cases of rare tumors and a review of the literature. Crit Rev Oncol Hematol. 2013 May;86(2):191-9.
  11. Chowdhry M, Hughes C, Grimer RJ, et al. Bone sarcomas arising in patients with neurofibromatosis type 1. J Bone Joint Surg Br. 2009 Sep;91(9):1223-6.
  12. Botto LD, Flood T, Little J, et al. Cancer risk in children and adolescents with birth defects: a population-based cohort study. PLoS One 2013;8(7):e69077.
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