Published online by Cambridge University Press: 05 November 2015
Soft tissue sarcoma
Soft tissue sarcomas are mesenchymally derived malignant tumours that may arise virtually anywhere in the body. They are rare, with an incidence of 45 per million (http://www.cancerresearchuk.org/, accessed January 2015). The overall survival is approximately 50% at 5 years. Treatment is largely surgical with adjuvant radiotherapy offered where there is deemed to be a significant risk of local relapse. Of patients with intermediate or high-grade tumours, 50% develop metastatic disease, usually by haematogenous spread to the lungs or in the case of sarcomas draining to the portal vein, the liver. Palliative chemotherapy may be offered, usually with single agent doxorubicin. Response rates are low at around 20%, although a further 20% achieve disease stabilisation. UK and ESMO European guidance on the management of soft tissue sarcoma tumours exist (Grimer et al., 2010b; The ESMO and European Sarcoma Network Working Groups, 2012b). American NCCN Guidelines are also available after free registration at the NCCN website. The National Institute for Health and Clinical Excellence has published Improving Outcomes Guidance for people with sarcoma (NICE, 2006).
Types of soft tissue tumour
Over 100 different morphology codes relating to sarcoma are present within the ICD-O-3 and World Health Organisation (WHO) classifications of sarcomas, but they can be grouped into the subtypes leiomyosarcoma, liposarcoma, fibrosarcoma, synovial sarcoma, vascular sarcoma, nerve sheath tumours, malignant phylloides tumours, sarcoma not-otherwise-specified and others. This chapter will not deal with GIST, which is covered in Chapter 18, rhabdomyosarcoma (covered in Chapter 40), and Kaposi's sarcoma (in Chapter 36). Ewing sarcoma of soft tissue and bone are treated identically and is covered in the bone sarcoma section of this chapter.
Incidence and epidemiology
A detailed analysis of the epidemiology of soft tissue sarcoma in England has been published recently by the West Midlands Cancer Intelligence Unit and is available online as ‘Soft Tissue Sarcoma Incidence and Survival Tumours Diagnosed in England Between 1985 and 2009’ (Dennis et al., 2012). The age-standardised soft tissue sarcoma incidence rates in England is around 45 cases per million, similar to the rates found in other registries outside the UK.