Soft Tissue Sarcoma Knowledge Centre
Understanding
Epidemiology
Prevalence
Soft Tissue Sarcomas (STS) are rare, with an incidence of 4-6 new cases/100,000/year; and some of the subtypes are even rarer, with less than 50 new cases each year. The aetiology of the disease is not known although several predisposing genetic abnormalities have been identified.
STS account for approximately 1% of all adult cancers1. In the European Union, 5,000-15,000 people are diagnosed with STS and 3,000-4,000 die of the disease every year2. The incidence of STS appears to be increasing3, possibly as a result of better recognition and diagnosis.
Exposure to ionising radiation is a well-established extrinsic risk factor for the disease. Sarcomas often develop at the site of radiation. This holds true whatever the part of the body affected, though most studies have investigated sarcomas developing after breast cancer treatment. Chronic lymphoedema has also been identified as a major risk factor, whereas exposure to certain toxic substances has also been shown to increase the risk of developing sarcomas. Finally, retrospective studies frequently mention a history of previous injuries as a possible triggering factor for the disease4.
References:
1. Jemal A, Siegel R et al. (2007) “Cancer statistics, 2007.” CA Cancer J Clin 57: 43–66.
2. Leyvraz S. (2007) “Soft tissue sarcomas: ESMO Clinical Recommendations for diagnosis, treatment and follow-up.” Ann Oncol 18(Suppl 2): ii74–76.
3. Zahm SH, Fraumeni Jr JF et al. (1997) “The epidemiology of soft tissue sarcoma.” Semin Oncol 24(5): 504–14.
4. Morgan JA, Le Cesne A, Chawla S, et al. Randomized phase II study of trabectedin in patients with liposarcoma and leiomyosarcoma (L-sarcomas) after failure of prior anthracylines (A) and ifosfamide (I). J Clin Oncol. 2007 Jun 20;25(18S):10060.