title: UK guidelines for the management of soft tissue sarcomas.
abstract: Soft tissue sarcomas (STS) are rare tumours arising in mesenchymal tissues, and can occur almost anywhere in the body. Their rarity, and the heterogeneity of subtype and location means that developing evidence-based guidelines is complicated by the limitations of the data available. However, this makes it more important that STS are managed by teams, expert in such cases, to ensure consistent and optimal treatment, as well as recruitment to clinical trials, and the ongoing accumulation of further data and knowledge. The development of appropriate guidance, by an experienced panel referring to the evidence available, is therefore a useful foundation on which to build progress in the field. These guidelines are an update of the previous version published in 2010 (Grimer et al. in Sarcoma 2010:506182, 2010). The original guidelines were drawn up following a consensus meeting of UK sarcoma specialists convened under the auspices of the British Sarcoma Group (BSG) and were intended to provide a framework for the multidisciplinary care of patients with soft tissue sarcomas. This current version has been updated and amended with reference to other European and US guidance. There are specific recommendations for the management of selected subtypes of disease including retroperitoneal and uterine sarcomas, as well as aggressive fibromatosis (desmoid tumours) and other borderline tumours commonly managed by sarcoma services. An important aim in sarcoma management is early diagnosis and prompt referral. In the UK, any patient with a suspected soft tissue sarcoma should be referred to one of the specialist regional soft tissues sarcoma services, to be managed by a specialist sarcoma multidisciplinary team. Once the diagnosis has been confirmed using appropriate imaging, plus a biopsy, the main modality of management is usually surgical excision performed by a specialist surgeon. In tumours at higher risk of recurrence or metastasis pre- or post-operative radiotherapy should be considered. Systemic anti-cancer therapy (SACT) may be utilized in some cases where the histological subtype is considered more sensitive to systemic treatment. Regular follow-up is recommended to assess local control, development of metastatic disease, and any late-effects of treatment. For local recurrence, and more rarely in selected cases of metastatic disease, surgical resection would be considered. Treatment for metastases may include radiotherapy, or systemic therapy guided by the sarcoma subtype. In some cases, symptom control and palliative care support alone will be appropriate.
authors.affiliation: Bristol Cancer Institute, Bristol Haematology & Oncology Centre, University Hospitals Bristol NHS Trust, Bristol, BS2 8ED UK.
authors.forename: Adam
authors.identifier: 0000-0003-3533-6842
authors.initials: A
authors.lastname: Dangoor
authors.affiliation: Department of Oncology, University College London Hospital NHS Trust, London, NW1 2PG UK.
authors.forename: Beatrice
authors.initials: B
authors.lastname: Seddon
authors.affiliation: The Newcastle upon Tyne Hospitals NHS Foundation Trust, Freeman Hospital, Newcastle-upon-Tyne, NE7 7DN UK.
authors.forename: Craig
authors.initials: C
authors.lastname: Gerrand
authors.affiliation: Royal Orthopaedic Hospital NHS Trust, Birmingham, B31 2AP UK.
authors.forename: Robert
authors.initials: R
authors.lastname: Grimer
authors.affiliation: Department of Oncology, University College London Hospital NHS Trust, London, NW1 2PG UK.
authors.forename: Jeremy
authors.initials: J
authors.lastname: Whelan
authors.affiliation: Royal Marsden NHS Foundation Trust, London, SW3 6JJ UK.
authors.forename: Ian
authors.initials: I
authors.lastname: Judson