Sarcomas are rare malignant tumours of bone and soft tissue. They are a heterogeneous group of malignancies, and include many anatomical sites and subtypes. There are approximately 850 new cases of sarcoma each year in Australia.
Increasingly, new prognostic factors and therapeutic approaches for sarcoma are being identified. However, the rarity of sarcoma and its sub-types makes it challenging to determine optimal treatment strategies. In addition, there are significant gaps in the evidence base used to underpin clinical decision making for patients with sarcoma.
This project was commenced as a collaboration between the Australasian Sarcoma Study Group (ASSG) and Cancer Council Australia (CCA) in 2011. The aims of the project were to appraise the available evidence guiding the management of patients with bone and soft tissue sarcoma, with an emphasis on Australian experience, access to specialist centres and facilities.
The process has been invaluable in bringing lead clinicians managing sarcoma, across a range of disciplines, to develop common shared understanding of the current evidence and to identify key research gaps. Development of pathways of care, both state and national, is a natural sequelae to this process.
The working party were asked to decide on the questions that were most relevant to their disciplines. The selected questions reflected the gaps in knowledge that impacted most on daily management decisions. As an ab initio set of guidelines, the original scope of these guidelines was broad. The key areas covered have been refined to include:
- Multidisciplinary treatment
- Chemotherapy (Systemic therapies)
The Wiki Guidelines platform that was used to develop these guidelines is unique. It enables iterative, ongoing and interactive guideline development. The Wiki will facilitate monitoring and assessing literature updates and allows guideline content to be updated instantly when required. In this way, the guidelines become a ‘living document’ which reflect the latest evidence available.
The working party recognise that sarcomas affect children and adolescents, as well as adult members of the community. However, for reasons of pragmatism and resource, the scope of this first iteration is restricted to adult bone and soft tissue sarcoma. Gastrointestinal stromal tumours (GIST), Kaposi’s sarcoma and desmoid fibromatosis were excluded. It is anticipated that next iteration of these guidelines will also include childhood, adolescent (AYA) and gynaecological sarcomas.
There were specific challenges encountered during the development of these guidelines, as low levels of evidence often underpin clinical sarcoma practice. Balanced with this is a pragmatic requirement for clinicians to make decisions on the optimal management of their individual patients. Nonetheless, these guidelines are based on a systematic review and a rigorous appraisal process, rather than a ‘consensus document’. A separate consumer guideline has not been developed. This decision reflects the complexity of sarcoma, as well as the availability of ‘on line’ consumer resources within Australia and the international community. Links to consumer resources have been provided in the “Information for consumers” section.
Sarcomas have traditionally been managed by wide excisional surgery and radiotherapy; with the use of chemotherapy reserved for advanced disease. Advances in multidisciplinary care have improved the evaluation and care of patients with this disease. In addition to paradigm shifts towards for example, limb-conserving surgery, there have been developments in other key modalities including radiotherapy techniques and novel therapeutic agents for specific tumor subtypes. Accurate pretreatment evaluation is therefore critical, particularly in planning multimodal treatment with curative intent, and also in providing ‘best practice’ treatment for advanced disease.
These guidelines highlight the importance of early sarcoma referral to multidisciplinary centers that specialise in treating this disease. Caseload and experience is associated with improved rates of functional limb preservation, lower rates of local recurrence, good rates of overall survival and improved quality of life.
The importance of the multidisciplinary team in initial assessment, diagnosis and making decisions about treatment is strongly endorsed by these recommendations. A multidisciplinary approach (involving pathologists, radiologists, surgeons, radiation therapists, medical oncologists and paediatric oncologists, with experience in sarcoma), or within reference networks sharing expertise and treating a high number of patients annually is preferred. These centres are usually involved in ongoing clinical trials, in which sarcoma patients' enrollment is highly encouraged.
This centralised referral should be pursued as early as at the time of the clinical diagnosis of a suspected sarcoma. The importance of appropriate diagnosis, including biopsy, review by an experienced histopathologist, and determination of grade and subtype to preoperative planning –particularly preoperative radiotherapy – cannot be underestimated.
In practice, referral of all patients with a lesion likely to be a sarcoma would be recommended. This would mean referring all patients with an unexplained deep mass of soft tissues, or with a superficial lesion of soft tissues having a diameter of >5 cm (or arising in childhood).
The draft guidelines containing 54 recommendations and 35 practice points were released for initial public consultation for a 30 day period on 3 September 2013. The consultation process involved soliciting public comments by sending email alerts to recipients comprising relevant professional organisations, state and territory Cancer Councils and individual clinical experts and consumer organisations in Australia and New Zealand, and inviting them to post their comment on the Cancer Council Australia Cancer Guidelines Wiki. During the public consultation phase nine public comments were received. These led to further edits, which were reviewed in detail by the working party.
We hope these guidelines will provide accessible up-to-date research for multidisciplinary sarcoma teams, individual clinicians, students and consumers. There are significant gaps in the evidence however, these are included where possible, in the form of future research questions that need to be addressed by good quality collaborative trials.
A key outcome of this process has been the need to develop more formal communications between sarcoma centres and clinicians, particularly in relation to current trials, and access.
This is the first step towards more standardised care for patients with sarcoma across the nation and provides a framework to educate the community about specialist pathways and to enhance inter-group collaboration.
I would like to thank my colleagues on the working party who gave voluntarily of their time to undertake the challenging task of appraising the evidence, writing the recommendations and meeting the guideline development deadlines. I thank the CEO of Cancer Council Australia, Professor Ian Olver for his support to this process. I also thank Cancer Council Australia’s project team; without the professionalism, drive and persistence of Christine Vuletich, Jutta von Dincklage, Laura Holliday these guidelines would never have been.
Chair, Sarcoma Guidelines Working Party
On behalf of the Australasian Sarcoma Study Group, Cancer Council Australia and the Working Party
- Clinical Guidelines Network Cancer Council Australia. Development of Clinical Practice Guidelines using Cancer Council Australia’s Cancer Guidelines Wiki. Handbook for section authors and the guideline working party. CCA Sydney; 2014 Available from: http://wiki.cancer.org.au/australiawiki/images/9/9b/CCA_Clinical_Practice_Guideline_Development_Handbook.pdf.