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.

Incidence, distribution of histological subtypes and primary sites of soft tissue sarcoma in China

“…The rate in Austria showed similar trend but peaked and dropped at younger ages 14 . Unlike carcinomas, STSs comprise a large proportion of pediatric malignancies and are an important cause of death in adolescents and young adults 3,4 . In our study, 3.1% of all STSs occurred in individuals aged 0 to 19 years, whereas in United States and Japan, corresponding proportions were 5.6% and 6.7%, respectively 3,12 .…”

“…The rarity of STSs and the even smaller number of cases in each combination further add to the difficulty faced by researchers and physicians. Although the national survival of STSs in China is still unknown, overall five-year survival rate of STSs in developed countries ranged from 55% to 78% 4,8,30 , indicating a relatively optimistic prognosis. Nevertheless, survival may vary tremendously (48.0%-99.5%) between different subtypes 30 , and would worsen along with ageing 3 .…”

“…Nevertheless, according to a European study, nearly 42% of the first histological diagnoses of STSs were inaccurate. This heterogeneity and discrepancy can lead to misreport on subtype incidence 4,32 and consequently bias the results on the epidemiology of STSs. Second, proportion of sarcoma NOS was too high compared with Japanese results 12 , and MV% was lower than that in Europe 6 , indicating a necessity to strengthen the pathological diagnosis especially in rural China.…”

“…Compared with other common cancer types, STSs can develop at almost any anatomical sites 2 , and are more prone to occur at childhood 3 . Although the etiology of STSs is still unclear, known risk factors include inherited syndromes, chemical and radiation exposures, viral infections and genetic mutations 4,5 . …”

“…In Europe, nearly 23,600 new STS cases rose annually and the crude incidence rate was 4.7 per 100,000 6 . In UK alone 3,300 new cases were diagnosed each year, with about 90 cases in children under 15 years oldd 4 . So far, most national and subnational epidemiological researches on the burden of STSs were carried out in developed countries 3,8-15 .…”