UK guidelines for the management of soft tissue sarcomas

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.     

Clinical practice guidelines: 2006 update of recommendations for the radiotherapeutic management of patients with soft tissue sarcoma (sarcoma of the extremity, uterine sarcoma and retroperitoneal sarcoma]

CONTEXT: The National French Federation of Comprehensive Cancer Centres (FNCLCC) initiated the update of clinical practice guideline for the management of patients with soft tissue sarcoma in collaboration with the French Sarcoma Group (GSF-GETO), specialists from French public universities, general hospitals and private clinics and with the French National Cancer Institute. This work is based on the methodology developed in the "Standards, Options and Recommendations" (SOR) project. OBJECTIVES: To update SOR guidelines for the management of patients with soft tissue sarcoma previously validated in 1995. METHODS: The methodology is based on a literature review and critical appraisal by a multidisciplinary group of experts who define the CPGsaccording to the definitions of the Standards, Options and Recommendations project. Once the guidelines have been developed, they are reviewed by independent reviewers. RESULTS: This article presents the updated recommendations for radiotherapeutic management. The main recommendations are: 1) irradiation before or after surgical treatment is the standard for soft tissue sarcoma of the extremity and uterine sarcoma; 2) no systematic irradiation should be done in case of retroperitoneal sarcoma.     

Intérêt des banques de tumeurs virtuelles annotées dans les sarcomes : expérience et projet du Groupe Sarcome Français et du réseau européen Conticanet

Sarcomas are rare and various tumours likely to constitute an adequate model for collaborative studies of collected data and tumour samples. The French Sarcoma Group has created an “annotated virtual tumour bank” that includes currently 3900 cases. This tumour bank is one of the main activities of the expert network on sarcoma biopathology supported by the National Institute of Cancer. An ongoing project consists in the organization of a similar tumour bank on sarcomas, shared by all European countries via the web and supported by the network of excellence Conticanet.     

Bone Tumors in Adolescents and Young Adults

Opinion statement  Bone tumors, particularly osteosarcomas and members of the Ewing Sarcoma Family of Tumors (ESFT), are typical malignancies of adolescents and young adults. Current diagnostic and therapeutic guidelines for patients of all ages were developed in this specific age group. The aim of bone sarcoma therapy should be to cure the patient from both the primary tumor and all (micro-)metastatic deposits while maintaining as much (extremity) function and causing as few treatment-specific late effects as possible. Bone sarcoma therapy requires close multidisciplinary cooperation. Usually, it consists of induction chemotherapy, followed by local therapy of the primary tumor (and, if present, primary metastases) and further, adjuvant chemotherapy. Local treatment for osteosarcoma should be surgery whenever feasible. Surgery is also gaining importance in ESFT, which was long considered a domain of radiotherapy. Modern reconstructive techniques continue to expand the indications for limb salvage, particularly for patients who have not yet reached skeletal maturity. Treatment within the framework of prospective, multi-institutional trials should be considered standard of care not only for children, but also for affected adolescents and (young) adults. Such trials are essential in guaranteeing that all patients have access to appropriate care and that progress from biological studies can be translated into prognostic improvements without undue delay. The rarity of bone sarcomas increasingly requires trials to be multinational.     

Nasty or Nice? Findings from a UK Survey to Evaluate the Impact of the National Institute for Health and Clinical Excellence (NICE) Clinical Guidelines on the Management of Prostate Cancer

AimsAlthough the National Institute for Health and Clinical Excellence clinical guideline 58 (CG58) for prostate cancer management was expected to have a positive effect, several recommendations raised concern among UK physicians. We conducted a survey of UK oncologists in 2008 and a second, similar survey in 2010 to assess views on these recommendations and to evaluate the change in opinion over time.Materials and methodsTwo semi-structured questionnaires were issued by the British Uro-oncology Group to society members in September 2008 and October 2010.ResultsIn 2008, 61 UK oncologists completed the survey; 60% agreed that CG58 would make a positive contribution towards improving patient care. There was strong opposition towards active surveillance as the first-line treatment for men with low-risk localised prostate cancer (49% disagreement); implementing 5 yearly flexible sigmoidoscopy post-prostate radiotherapy (51% disagreement); offering follow-up outside of the hospital (e.g. by general practitioners in primary care) for men with a stable prostate-specific antigen for ≥2 years (44% disagreement); and recommendations against docetaxel retreatment (47% disagreement) or bisphosphonate use (58% disagreement). In 2010, 77 UK oncologists completed the survey. The results were largely consistent with 2008, although several recommendations, particularly for localised disease, seem to have promoted a change in clinical practice, suggesting that they are facilitating a standardised approach. Compared with 2008, the 2010 results indicate a shift in favour of active surveillance (80% agreement) and primary care follow-up (59% agreement), but increasing opposition for docetaxel retreatment (57% disagreement). Opinions remained divided for flexible sigmoidoscopy and bisphosphonates.ConclusionsDespite initial concerns, the CG58 seems to have had a positive impact on prostate cancer management in the UK, with adherence likely facilitating a standardised approach. However, with new data emerging, these findings underscore the need to regularly update guidelines. A revision of the CG58 is anticipated by 2014.     

Clinical practice guidelines for the psychosocial care of adults with cancer

Clinical practice guidelines are increasingly being developed in medical settings to provide evidence-based recommendations to guide the clinical care of patients. The development of Clinical practice guidelines for the psychosocial care of patients with medical illness is a newer initiative, and more complex as the target audience includes health care professionals from diverse backgrounds. In Australia, the National Breast Cancer Centre and National Cancer Control Initiative have collaborated to develop Clinical practice guidelines for the psychosocial care of adults with cancer, funded by the Australian Government Department of Health and Ageing. This paper outlines the development of these guidelines in the international context, gives an overview of their content, and describes strategies for their implementation and evaluation.     

乳腺癌患者骨质疏松非药物干预的最佳证据总结

目的 检索、提取并汇总乳腺癌患者骨质疏松非药物干预的最佳证据。方法 检索UpToDate、BMJ、JBI、国际指南协作网、英国国家骨质疏松指南组、Cochrane、PubMed、医脉通、中国知网、万方医学网等网站及数据库中关于乳腺癌患者骨质疏松非药物干预相关措施的临床决策、指南、专家共识和系统评价。检索时间限定为2017年1月1日至2022年11月8日,由2名研究员独立对文献进行质量评价。结果 共纳入14篇文献,其中临床决策4篇,指南4篇,专家共识5篇,系统评价1篇,最终围绕乳腺癌患者骨质疏松的筛查与评估、非药物干预措施及注意事项3个维度汇总了25条最佳证据。结论 乳腺癌患者骨质疏松非药物干预的证据有助于临床医护人员开展骨健康管理,为指导乳腺癌患者进行骨质疏松非药物管理提供循证依据。     

Traitements locorégionaux des sites métastatiques chez des patients atteints d’un mélanome pauci-métastatique (hors métastase cérébrale) : recommandations nationales françaises

Introductionthe last years are marked by the emergence of new molecules for the treatment of metastatic cutaneous melanoma with a significant benefit on the survival. Besides, some techniques are in development for the loco-regional treatment of the metastatic sites, bringing new therapeutic perspectives. However, their respective use and place in the therapeutic strategy are debated by healthcare professionals.Objectivethe French National Cancer Institute leads a national clinical practice guidelines project since 2008. It realized a review of these modalities of treatment and developed recommendations.Methodsthe clinical practice guidelines development process is based on systematic literature review and critical appraisal by a multidisciplinary expert workgroup. The recommendations are thus based on the best available evidence and expert agreement. Prior to publication, the guidelines are reviewed by independent practitioners in cancer care delivery.Resultsthis article presents recommendations for loco-regional treatments of the pulmonary, bone, cutaneous, hepatic and digestive metastatic sites for patients with pauci-metastatic cutaneous melanoma.     

Development of guidelines for distress management in Korean cancer patients

Objectives: Psychological distress is common in cancer patients, and the need to develop a system for assessing and managing distress is widely recognized. This project developed recommendations that are feasible for Korean cancer patients and the Korean healthcare system. Methods: Based on the findings from a series of studies in the context of this project, we developed guidelines following the steps and parameters recommended by the Scottish Intercollegiate Guidelines Network (SIGN). The Development Group consisted of individuals from several professions, including psychiatrists, psychologists, nurses, social workers, a health policy expert, and a methodologist. Opinions from various healthcare providers, patients, and related societies were also reflected in the guidelines. Results: The main recommendations for distress management in cancer patients were the following: (1) a concept of distress in Korean cancer patients, screening tools, management algorithms, and triage approaches was developed and (2) four symptom‐specific guidelines with management algorithms were proposed for depression, anxiety, insomnia, and delirium. Conclusion: This is the first effort to develop recommendations for distress management in psycho‐oncology in Korea. These guidelines offer standards for psychosocial care for cancer patients in Korea. We have made a significant step toward integrated cancer care that incorporates the psychosocial care of patients as an essential component of patient care in a Korean oncology context. This version will be updated constantly to keep up with emerging evidence from empirical research and clinical experience. Copyright © 2011 John Wiley & Sons, Ltd.     

Consensus recommendations for the use of anti-egfr therapies in metastatic colorectal cancer

In January 2010, a panel of Canadian oncologists with particular expertise in colorectal cancer (crc) gathered to develop a consensus guideline on the use of therapies against the epidermal growth factor receptor (egfr) in the management of metastatic crc (mcrc). This paper uses a case-based approach to summarize the consensus recommendations developed during that meeting.These are the consensus recommendations:Testing for the KRAS status of the tumour should be performed as soon as an egfr inhibitor is being considered as an option for treatment.Anti-egfr therapies are not recommended for the treatment of patients with tumours showing mutated KRAS status.For a patient with wild-type KRAS and an Eastern Cooperative Oncology Group status of 0-2, whose mcrc has previously been treated with a fluoropyrimidine, irinotecan, and oxaliplatin, switching to an egfr inhibitor is a recommended strategy.Cetuximab, cetuximab plus irinotecan, and panitumumab are all options for third-line therapy in patients with wild-type KRAS, provided that tolerability is acceptable.     

Summary and comparison of myeloid growth factor guidelines in patients receiving cancer chemotherapy

Three different practice guidelines for the myeloid growth factors were recently published by major professional organizations. A comprehensive review and comparison of the guidelines using a priori structured content criteria and a previously validated quality appraisal tool are reported. The final recommendations from these guidelines are consistent for primary prophylaxis with the colony-stimulating factors (CSFs) when the risk of febrile neutropenia is in the range of 20% or greater. All 3 guidelines also recommend prophylactic use of myeloid growth factors in patients with important factors increasing individual risk of neutropenic complications. The recommendation that patients receiving regimens associated with lower risk should have CSF use guided by individual risk assessment is also consistent. Critical quality appraisal indicates that the scope, purpose, stakeholder involvement, and applicability of these guidelines differ little. The American Society of Clinical Oncology and European Organization for Research and Treatment of Cancer guidelines place more emphasis on comprehensive literature reviews, and the National Comprehensive Cancer Network guidelines are more current based on systematic annual updates. Presentation clarity also favors National Comprehensive Cancer Network guidelines, with recommendations generally presented as both text and algorithmic diagram.     

Guidelines for the adjuvant treatment of postmenopausal women with endocrine-responsive breast cancer: past, present and future recommendations

Treatment guidelines are useful tools that enable physicians to integrate the latest clinical research into their practices. The large volume of rapidly evolving clinical data in breast cancer has been summarised and incorporated into treatment recommendations by well-known and reliable institutions, including the National Comprehensive Cancer Network, the American Society for Clinical Oncology, the European Society for Medical Oncology and the St. Gallen International Consensus Panel. Adjuvant therapy is a key component of breast cancer treatment, and many of the current consensus guidelines now recognise the important role of the aromatase inhibitors as an alternative to or in sequence after tamoxifen, hitherto the standard adjuvant treatment of choice for receptor-positive women. Data from ongoing trials such as the Breast International Group 1-98 trial and those still in the accrual phase will be forthcoming and will likely result in a further refinement of treatment recommendations over the course of the next few years. Despite the availability of such guidelines, however, there is evidence that adherence to and implementation of treatment recommendations is less than optimal. Further research is needed to determine more effective means of disseminating those clinical recommendations that can have a significant impact on treatment strategies and ultimately improve outcomes in breast cancer.     

An update on the management of sporadic desmoid-type fibromatosis: a European Consensus Initiative between Sarcoma PAtients EuroNet (SPAEN) and European Organization for Research and Treatment of Cancer (EORTC)/Soft Tissue and Bone Sarcoma Group (STBSG)

Desmoid-type fibromatosis is a rare and locally aggressive monoclonal, fibroblastic proliferation characterized by a variable and often unpredictable clinical course. Currently, there is no established or evidence-based treatment approach available for this disease. Therefore, in 2015 the European Desmoid Working Group published a position paper giving recommendations on the treatment of this intriguing disease. Here, we present an update of this consensus approach based on professionals’ AND patients’ expertise following a round table meeting bringing together sarcoma experts from the European Organization for Research and Treatment of Cancer/Soft Tissue and Bone Sarcoma Group with patients and patient advocates from Sarcoma PAtients EuroNet. In this paper, we focus on new findings regarding the prognostic value of mutational analysis in desmoid-type fibromatosis patients and new systemic treatment options.     

Recommendations of a group of experts for the pathological assessment of tumour regression of liver metastases of colorectal cancer and damage of non-tumour liver tissue after neoadjuvant therapy

Colorectal cancer (CRC) incidence has increased during the past decades in Spain, being the first malignant tumour in incidence. Observed mortality for CRC is mainly due to liver and lung metastases. The only curative treatment is surgery; new surgical techniques and neoadjuvant treatments have increased the number of surgery candidate patients. Patients should be managed with a multidisciplinary approach that includes imaging techniques, chemotherapy, surgery and pathological assessment. As an answer to this approach, a group of pathology experts interested on CRC liver metastases aimed to review the diagnosis and prognosis of liver mestastases and developed practical recommendations for its assessment. The expert group revised the current literature and prepared questions to be discussed based on available evidence and on their clinical practise. As a result, recommendations for the assessment of tumour regression of liver metastases are proposed, which could be implemented in oncology centres allowing assessment standardisation for these patients. Prospective multi-center studies to evaluate these recommendations validity will further contribute to improve the standard care of CRC liver metastases patients.     

Guidelines for surgical treatment of hepatoblastoma in the modern era--recommendations from the Childhood Liver Tumour Strategy Group of the International Society of Paediatric Oncology (SIOPEL)

Cisplatin-containing chemotherapy and complete surgical resection are both crucial in the cure of hepatoblastoma. Radical resection can be obtained either conventionally by partial hepatectomy or with orthotopic liver transplant, but the surgical approach to hepatoblastoma differs considerably across the world. Our main aim in this paper is to present the surgical recommendations of the Childhood Liver Tumour Strategy Group of the International Society of Paediatric Oncology (SIOPEL), as well as to stimulate international debate on this issue. We discuss biopsy, verification of resectability, resection principles, indications and potential contraindications for orthotopic liver transplant, as well as thoracic surgery for pulmonary metastases. We suggest that heroic liver resections with a high probability of leaving residual tumour should be avoided whenever possible. In such cases primary orthotopic liver transplant should be considered. Superior survival rates in hepatoblastoma patients who have received a primary transplant after a good response to chemotherapy support the strategy of avoiding partial hepatectomy in cases where radical resection appears difficult and doubtful. We recommend early referral to a transplant surgeon in cases of: (i) multifocal or large solitary PRETEXT IV (PRE Treatment EXTent of disease scoring system) hepatoblastoma involving all four sectors of the liver and (ii) unifocal, centrally located tumours involving main hilar structures or main hepatic veins. Because complete tumour resection is a prerequisite for cure, any strategy leading to an increased resection rate will result in improved survival. We advise the more frequent use of orthotopic liver transplant, as well as the standardisation of techniques for partial liver resection. These guidelines should not be seen as final, but rather as a starting point for further discussion between the various national and international liver tumour study groups.     

Dose-intensive chemotherapy with growth factor or autologous bone marrow or stem-cell transplant support in first-line treatment of advanced or metastatic adult soft tissue sarcoma: a clinical practice guideline

Questions

• In patients with inoperable locally advanced or metastatic soft tissue sarcoma, does first-line dose-intensive chemotherapy supported by growth factor or autologous bone marrow or stem-cell transplantation improve response rate, time to disease progression, or survival as compared with standard-dose chemotherapy?
• What are the effects of first-line dose-intensive chemotherapy supported by growth factor or autologous bone marrow or stem-cell transplantation on toxicity and quality of life?

Perspectives

Because therapeutic options for adult patients with advanced or metastatic soft tissue sarcoma are scarce and the possibility of cure for these patients is extremely limited, the Sarcoma Disease Site Group (dsg) felt that a review of the available literature on dose-intensive chemotherapy for adult patients with locally advanced or metastatic soft tissue sarcoma and subsequent development of a clinical practice guideline based on the evidence were important.

Methodology

A systematic review was developed and clinical recommendations relevant to patients in Ontario were drafted. The practice guideline report was reviewed and approved by the Sarcoma dsg, which comprises medical oncologists, radiation oncologists, surgeons, a pathologist, a methodologist, and community representatives. External review by Ontario practitioners was obtained through a mailed survey, the results of which were incorporated into the practice guideline. Final review and approval of the practice guideline was obtained from the Report Approval Panel.

Practice Guideline

Based on the systematic review, consensus, and external review, the Sarcoma dsg makes these recommendations:

• Dose-intensive chemotherapy with growth factor support is not recommended in the first-line treatment of patients with inoperable locally advanced or metastatic soft tissue sarcoma.
• The data are insufficient to support the use of high-dose chemotherapy with autologous bone marrow or stem-cell transplantation as first-line treatment in this group of patients.
• Eligible patients should be encouraged to enter clinical trials assessing novel approaches or compounds.

Qualifying Statements

High-dose chemotherapy with growth factor or autologous bone marrow or stem-cell transplantation has adverse effects similar to those seen with standard-dose chemotherapy. With high-dose regimens, the incidence of grades 3 and 4 thrombocytopenia is significantly higher, and neutropenic fever and febrile neutropenia occur more frequently. Compared with standard treatment, the rate of treatment-related death is also higher with high-dose regimens.     

The Chicago Consensus on peritoneal surface malignancies: Standards

The Chicago Consensus Working Group provides the following multidisciplinary recommendations for the care of patients with peritoneal surface malignancies. This article focuses on the standards of a peritoneal surface malignancy center, standards of billing and coding, standards of operative reports for cytoreductive surgery and hyperthermic intraperitoneal chemotherapy, standards of cytoreductive surgery training, and standards of intraoperative chemotherapy preparation. These guidelines are developed with input from leading experts, including surgical oncologists, medical oncologists, pathologists, radiologists, palliative care physicians, and pharmacists. These guidelines recognize and address the emerging need for increased awareness in the appropriate management of peritoneal surface disease. They are not intended to replace the quest for higher levels of evidence.     

Standards, Options et Recommandations 2006. Prise en charge des patients adultes atteints de sarcome des tissus mous, de sarcome utérin ou de tumeur stromale gastro-intestinale

Context

The French National Federation of Comprehensive Cancer Centres (FNCLCC) initiated the update of clinical practice guidelines (CPGs) for the management of patients with soft tissue sarcoma, in collaboration with the French National Cancer Institute and the French Sarcoma Group (GSF-GETO), specialists from French public universities, general hospitals and private clinics. This work was based on the methodology developed by the Standards, Options and Recommendations (SOR) project.

Objectives

To update the SOR for the management of patients with soft tissue sarcoma, previously validated in 1995.

Methods

The methodology was based on a literature review and critical appraisal by a multidisciplinary group of experts who defined CPGs according to the definitions of the Standards, Options and Recommendations project. Once the guidelines were developed, they were reviewed by independent experts.

Results

Because of the difficulties inherent in diagnosing and treating sarcoma, it is essential to set up cooperative consulting for the management of these tumours. The updated SOR can serve as a tool to achieve that goal.