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33 - Management of soft tissue and bone tumours in adults
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- By Owen Tilsley, Velindre Cancer Centre, Velindre Hospital, Cardiff, UK
- Edited by Louise Hanna, Tom Crosby, Fergus Macbeth
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- Book:
- Practical Clinical Oncology
- Published online:
- 05 November 2015
- Print publication:
- 19 November 2015, pp 436-449
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Summary
Soft tissue sarcoma
Introduction
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.
40 - Management of cancer in children
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- By Owen Tilsley, Velindre Cancer Centre, Velindre Hospital, Cardiff, UK
- Edited by Louise Hanna, Tom Crosby, Fergus Macbeth
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- Book:
- Practical Clinical Oncology
- Published online:
- 05 November 2015
- Print publication:
- 19 November 2015, pp 538-550
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Summary
Introduction
This chapter aims to provide the reader with an introduction to the management of children with cancer. Contemporary reviews and resources will be referred to as appropriate. Radiotherapy is an important component of the treatment for many childhood tumours and this may involve advanced techniques including brachytherapy or protons, sometimes requiring referral to another centre or even abroad. Many different healthcare professionals are involved in the delivery of care, coordinated in the UK by specialised multidisciplinary teams (MDTs) at one of 19 principal treatment centres, often in collaboration with shared care units closer to home.
Paediatric cancer is uncommon. Of the 280,000 patients diagnosed with cancer in England in 2012, only 1303 were children (0.5%). The incidence of some childhood cancers has increased marginally over the last 40 years, but death rates have declined dramatically for all non-CNS childhood cancers. With cure rates now over 70%, the prevalence of paediatric cancer survivors in the population is increasing rapidly, posing challenges for the management of cancer survivorship and the late effects of treatment. The incidence of cancer between the ages of 10 and 65 is well modelled by a 10% increase per annum, which equates to a 10-fold increase every 25 years. Given the relative rarity of paediatric cancer, it is still unusual to encounter an adult survivor of paediatric malignancy, but will become more common.
An important challenge in treatment is to minimise toxicity while maximising the chance of cure, leading to a risk-based stratification of treatment intensity, including for radiotherapy. As a result, most paediatric cancer is delivered using nationally or internationally agreed protocols. Radiotherapy has significant late toxicity as it reduces the natural growth seen in childhood, and, because of the long life expectancy of survivors, it may cause radiotherapy-induced second malignancies.
The radiotherapy pathway
Giving radiotherapy to children is complex and requires a team of clinical oncologists, therapeutic radiographers, mould room staff and play specialists, together with nurses, anaesthetic staff, physicists, dosimetrists, and psychologists or psychotherapists.
Play therapy
Play therapy is important in preparing children for radiotherapy. If the trust and cooperation of a younger child can be gained, treatment may not require a general anaesthetic.
An atheloptic trilobite assemblage from the Carboniferous of North Devon
- R. M. Owens, J. W. Tilsley
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- Journal:
- Geological Magazine / Volume 132 / Issue 6 / November 1995
- Published online by Cambridge University Press:
- 01 May 2009, pp. 713-728
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New collections of trilobites have been made from Dinantian rocks in North Devon, distributed among seven genera. Of these, Wagnerispina, Archegonus (Phillibole), Liobole, Aprathia, and probably Lichanocoryphe gen. nov., are primitive phillipsiids belonging to the subfamily Archegoninae. Reasons are given here for the others, Tawstockia and Spatulina, previously considered to be primitive phillipsiids of the subfamily Cystispininae, to belong to the higher phillipsiids, as effaced, blind members respectively of the Linguaphillipsiinae and Cummingellinae. The species of these genera from North Devon discussed here, three of which are new, are either small-eyed or blind, and form an atheloptic assemblage; they occur in hemipelagic sediments of the Codden Hill Chert Group in the Barnstaple district, and the Kersdown Chert Formation in the Bampton district. Ammonoids associated with the trilobites indicate an early Viséan, late Chadian age for the fauna, and the same or similar trilobite species and subspecies occur in the Březina Shales of Moravia, suggesting that they are approximately coeval.
30 - Soft tissue and bone tumours in adults
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- By Owen Tilsley, Consultant, Clinical Oncologist, Velindre Cancer Centre, Velindre Hospital, Whitchurch, Cardiff, UK
- Edited by Louise Hanna, Tom Crosby, Fergus Macbeth
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- Book:
- Practical Clinical Oncology
- Published online:
- 23 December 2009
- Print publication:
- 24 January 2008, pp 335-346
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Summary
Introduction
Soft tissue sarcomas are rare. Although there are more than 30 subtypes, the behaviour of most is similar. Tumour size and grade are the best predictors of survival. Surgery is the mainstay of treatment, and adjuvant radiotherapy (RT) is useful for high-risk tumours. The use of adjuvant chemotherapy remains controversial, and metastatic disease responds poorly to doxorubicin and ifosfamide.
The most common bone cancer occurs as a secondary tumour, and the most common primary bone tumours are benign. Primary bone cancers are rare and include those derived from marrow elements, such as myeloma and lymphoma, which are discussed in Chapter 31. Osteosarcoma and Ewing's sarcoma make up the majority of bone sarcomas: they occur most commonly in people in their teens and 20s. Both types of tumour are treated with neoadjuvant and adjuvant ‘sandwich’ chemotherapy and local surgery. The role of RT in osteosarcoma is uncertain, but RT is important in the local control and cure of inoperable Ewing's sarcoma and as an adjunct to marginal surgery. Metastatic disease in both conditions carries a poor prognosis. Rarer bone sarcomas include chondrosarcoma, which responds poorly to either chemotherapy or RT. Other bone sarcomas that show morphological similarity to soft tissue sarcomas are treated in the same way as osteosarcomas. Aneurysmal bone cysts and giant cell tumours are of borderline malignancy.
Sarcomas of the uterus are discussed in Chapter 23 and gastrointestinal stromal tumours are discussed in Chapter 15. Sarcomas in children are discussed in Chapter 37.