Sarcoma Guidelines

  1. Preamble
  2. Executive Summary
  3. Plain Language Summary
  4. Topic 1: Treatment at Specialised Sarcoma Centre
  5. Topic 2: Retroperitoneal Sarcoma
  6. Topic 3: Paediatric/AYA Sarcoma
  7. Appendices

Preamble

As the peak professional organisation in sarcoma in Australia and New Zealand, the Australia and New Zealand Sarcoma Association (ANZSA) is dedicated to improving the outcome for patients with sarcoma through awareness, education, and research. ANZSA fosters effective collaborations across medical and psychosocial multidisciplinary team (MDT), comprising specialist health professionals (including pathologists, radiologists, surgeons, radiation oncologists, medical oncologists, paediatric oncologists, and allied health) working together to identify the best management and care of patients with sarcoma and related tumours. Pivotal to this goal is the development and dissemination of clinical practice guidelines based on systematic review to evaluate evidence for best management of adult and paediatric patients with sarcoma. This guidelines development process was commenced at the end of 2020 with full support from the board and the scientific advisory committee of ANZSA. The intended end users are health professionals involved in the care of patients with sarcoma from time of initial presentation through diagnosis, staging, treatment and follow up.

Sarcomas are rare and complex malignant tumours of bone and soft tissue. They are a heterogeneous group of malignancies and include many anatomical sites and pathological subtypes. Sarcoma affects both children and adults. In 2022, there were 2,460 new cases of sarcoma in Australia representing 1.5% of all new cancer diagnosis. In New Zealand, 193 cases of bone and soft tissue sarcoma were reported in 2019. Patients diagnosed with sarcoma had a 5-year survival of 69.3%. Optimal management of this complex malignancy requires MDT input that considers clinical, pathologic, and treatment factors. Due to the rarity of sarcoma and the diverse presentations, there are few randomised or prospective data presenting a specific challenge for the guidelines working party to develop evidence based recommendations.

ANZSA strives to avoid bias by ensuring the guidelines working party consists of a multidisciplinary group with variation in areas of clinical expertise, geographic region, gender, background, and practice setting (see Administrative Report for further details). The guidelines have been developed without commercial support, and members of the working party volunteer their time. The members are required to disclosure any potential conflicts of interest (see the Administrative Report for further details).                       

The working party identified three broad topics for the guidelines (Topic 1: management at specialised sarcoma centre; Topic 2: retroperitoneal sarcoma; Topic 3: paediatric/adolescent young adult (AYA) sarcoma), developed the priority questions and their patient/population, intervention, comparison, and outcome (PICO) models. A systematic review using the online Covidence systematic review management system was completed for each question. The lead authors for each outcome endpoint created evidence tables that summarised the findings for the working party to then appraise, formulate recommendations and practice points. For each evidence-based recommendation, the grade of recommendation is identified according to National Health and Medical Research Council (NHMRC) grades for recommendations (3).  Further details of the systematic review process are available in the Technical Report.

This current series of the guidelines focused on the following questions:

Topic 1: Treatment at specialised sarcoma centre

  1. Does radiotherapy at a specialised sarcoma centre improve outcomes?

  • Population: Adult and Paediatric patients with bone and soft tissue sarcoma 
  • Intervention: Multidisciplinary team, Radiotherapy at specialised centre
  • Comparison: Radiotherapy at non-specialised centre
  • Outcomes: Local control, overall survival, wound complication/toxicity
  1. Does surgery at a specialised sarcoma centre improve outcomes?
  • Population: Adult and Paediatric patients with bone and soft tissue sarcoma 
  • Intervention: Multidisciplinary team, Surgery at specialised centre
  • Comparison: Surgery at non-specialised centre
  • Outcomes: Local control, limb conservation rate, 30-day and 90-day surgical mortality, overall survival

Topic 2: Retroperitoneal Sarcoma

  1. What is the role of preoperative biopsy in the management of primary retroperitoneal sarcoma?

  • Population - Adult patients with retroperitoneal sarcoma
  • Intervention – Preoperative biopsy
  • Comparator – no biopsy
  • Outcomes – biopsy tract seeding, recurrence free survival, overall survival
  1. Does multi-visceral resection improve outcomes for patients with primary retroperitoneal sarcoma?
  • Population - Adult patients with primary localised retroperitoneal sarcoma
  • Intervention - multi-visceral resection (including adjacent organs uninvolved on preoperative imaging)
  • Comparator – simple surgical resection
  • Outcomes – abdominal recurrence free survival, recurrence free survival, perioperative morbidity, overall survival 
  1. What is the role of radiation therapy in the management of primary retroperitoneal sarcoma?
  • Population - Adult patients with primary retroperitoneal sarcoma
  • Intervention – Surgical resection with neoadjuvant or adjuvant radiation therapy
  • Comparator – Surgical resection alone
  • Outcomes – abdominal recurrence free survival, recurrence free survival perioperative morbidity, overall survival
  1. What is the role of chemotherapy in the management of primary retroperitoneal sarcoma?
  • Population - Adult patients with primary retroperitoneal sarcomas
  • Intervention – Surgical resection with neoadjuvant or adjuvant chemotherapy
  • Comparator – Surgical resection without chemotherapy
  • Outcomes – Recurrence free survival, overall survival, post-operative complications

Topic 3: Paediatric and AYA Sarcoma

  1. Does delayed surgical resection of the primary tumour impact on outcome for patients with pelvic Ewing sarcoma?

  • Population: Localised pelvic Ewing Sarcoma
  • Intervention: Delayed surgical resection of the primary tumour
  • Comparison: Surgery at time point recommended for local control in protocol
  • Outcomes: Overall Survival
  1. Does the addition of high-dose chemotherapy have an impact on outcome of patients with Ewing sarcoma or rhabdomyosarcoma compared to standard chemotherapy alone? in first line (a)? In relapse (b)? 
  • Population: Ewing sarcoma and rhabdomyosarcoma
  • Intervention: high-dose/myeloablative chemotherapy with autologous stem cell rescue
  • Comparison: standard chemotherapy
  • Outcomes: Overall Survival, Event-Free Survival, Treatment Related Mortality and toxicity

The guidelines development is an ongoing process and recommendations for other key questions will be released in the future as the working party continues to appraise evidence for the rest of the topics. This series will be updated as new evidence becomes available and at a minimum of every five years.

The guidelines are applicable for both adult and paediatric patients with sarcoma in the Australian and New Zealand health care settings. The recommendations are relevant for all population subgroups of different social and cultural background.  It is recognised that the recommendation for management at specialised sarcoma centres which are only readily accessible in metropolitan locations can be challenging for rural patients and those from a lower socioeconomic background. It is hoped that the evidence generated from the systematic review can be leveraged to support these patients and their families.   

Angela Hong

Chair, on behalf of Clinical Practices Guidelines for Management of Sarcoma Working Party