Update of clinical practice guidelines for the management of patients with sarcoma

Sarcomas are rare and complex malignant tumours of bone and soft tissue with over 120 histologic subtypes. In 2023, there were 2249 new cases of soft tissue sarcoma and 276 cases of bone sarcoma in Australia representing 1.5% of all new cancer diagnoses.1 However they are the second most common cause of cancer-related death in the 0–19 age group.1 The heterogeneity of this rare group of cancers relies heavily on multidisciplinary expertise for optimal outcome. In May 2024, the Australian Federal Parliament Senate inquiry's final report on ‘Equitable access to diagnosis and treatment for individuals with rare and less common cancers, including neuroendocrine cancer’ was released. The inquiry acknowledged that patients with rare cancers do not always receive the same level of support or have access to the same diagnosis and treatment options, as those with more common cancers, with potentially significant deleterious consequences.2 The first of the 41 recommendations emphasized the need to develop guidelines and local pathways for rare cancers and to ensure that they are accessible for general practitioners at the point-of-care. This article summarizes the guidelines on topic 1 given the importance of timely referral of patients with suspected sarcoma to specialized sarcoma centres to ensure optimal outcomes. The evidence summary and recommendations for Topics 2 and 3 are shown in Table S1. The detailed method of systematic reviews of the guidelines questions has been published previously.3-8 In brief, literature searches for evidence were undertaken at various timepoints between 2021 and 2024 in the following electronic databases from 1990 onwards and limited to articles in English: Ovid Medline, Ovid Embase, Cochrane Central (Wiley). The title and abstract, followed by the full text of each study was assessed for eligibility per the population, intervention, comparison and outcome (PICO) model by two reviewers and reasons for exclusion assigned to each excluded study. The quality of each study was assessed using the NHMRC Evidence Hierarchy9 and the Newcastle-Ottawa Quality Assessment Form for Cohort Studies.10 The working party formulated the recommendations and the grade according to an NHMRC-approved method.9 The full details across the three topics are freely available on the ANZSA website (www.sarcoma.org.au). Plain language summaries translated into multiple languages are also available for consumers to improve their health literacy and empower them to make informed decision (Senate inquiry report on rare cancer recommendation 18, 4.114). Since the first publication of the guidelines on the website in July 2022, there have been 2931 page views of the landing page and 809 page views of the plain language summary page. ‘Does radiotherapy at a specialized sarcoma centre improve outcomes?’ with the PICO model as below: ‘Does surgery at a specialized sarcoma centre improve outcomes?’ with the PICO model as below: There were 66 retrospective studies identified in the systematic review and a large number of these studies from cancer registries.3 There was consistency in the literature demonstrating that patients who underwent surgery at specialized sarcoma centres (regardless of how a specialized centre was defined) had improved outcomes. Eleven studies sought to compare limb salvage rate based on case load. Six studies, with the largest one included over 14 000 patients from the United States, demonstrated an increased likelihood of limb salvage surgery compared to amputation in patients with primary bone tumours treated at high volume centres (OR 1.34, 95% CI 1.14–1.59 P = 0.001).14 Of the five studies that did not show a difference in limb salvage rates, three did not perform a multivariable analysis.15-17 Twelve studies reported on peri-operative mortality, with seven reporting a lower peri-operative mortality rate in high volume centres,18-24 The remaining five studies were likely underpowered to demonstrate a difference.25-29 Twenty-one studies assessed the overall survival endpoint comparing specialized with non-specialized sarcoma centres. Sixteen of these studies showed that surgery at a specialized centre was a significant predictor of improved overall survival.18-20, 22, 24, 30-40 The five studies which did not demonstrate a survival benefit were all of relatively low caseload and likely underpowered.41-45 Available evidences (level 3, 4) suggest that better local control, limb salvage, overall survival and lower 30-day and 90-day mortality when surgery at specialized sarcoma centres. The working party recommended that patients with suspected sarcoma be referred to a specialized sarcoma centre for management to reduce local recurrence and surgical complication, and to improve limb conservation and survival (Grade B).3 Patients with a suspected sarcoma should be managed through a specialized sarcoma centre. It is recognized that this can be challenging for rural patients as well as clinicians as there are only few specialized sarcoma centres in Australia and New Zealand. It is hoped that these evidence-based recommendations can be leveraged to advocate for equitable access to specialized sarcoma care for all patients and their families. The ANZSA guidelines development is ongoing with other clinical questions under review. We encourage users to refer to the ANZSA website (www.sarcoma.org.au) for full details of the guidelines. We would like to acknowledge the Australia and New Zealand Sarcoma Association for providing support to the development of these guidelines. We thank the consumer representatives of guidelines working party for their valuable contributions. Ganaps Perianayagam: Data curation, Data analysis, Writing - Review & Editing. Marianne Phillips: Conceptualization, Methodology, Data curation, Data analysis, Writing - Review & Editing. David Pryor: Conceptualization, Methodology, Data analysis, Writing - Review & Editing. Abay Sundaram: Data curation, Data analysis, Writing - Review & Editing. Stephen R Thompson: Conceptualization, Methodology, Data analysis, Writing - Review & Editing. Deborah Di-Xin Zhou: Data curation, Data analysis, Writing - Review & Editing. Angela M Hong: Conceptualization, Methodology, Software, Data curation, Data analysis, Writing - Original Draft, Writing - Review & Editing, Supervision. Table S1. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

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