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What is sarcoma?

Sarcoma is type of cancer arising in the bone or soft tissue (such as muscle, fat). It is a rare form of cancer representing about 1.3% of all cancer diagnoses in Australia and New Zealand. It can develop in many different parts of the body and there are many different types of sarcoma. The treatment may include surgery, radiation therapy, chemotherapy alone or in combination.

How were these guidelines developed?

These guidelines were developed by the Australia and New Zealand Sarcoma Association to inform the best management of patients with sarcoma using scientific evidence. The guidelines are based on a rigorous review of evidence by a group of independent experts (such as doctors, nurses and scientists) and consumer representatives (such as patients who have had sarcoma treatment and their carers). As sarcoma is rare, the available evidence is mostly from experiences from specialised sarcoma hospitals or cancer registry. There were no clinical trials found that addressed the three clinical questions in this series of the guidelines.

Referral to specialist sarcoma centre                                                   

The guidelines recommend patients be referred to specialised sarcoma hospitals where there is team of sarcoma experts who have regular meetings (multidisciplinary team meetings or 'MDT meetings') to discuss the best management for each individual patient with the diagnosis or suspected diagnosis of sarcoma. The scientific evidence suggests that outcomes are generally better when patients received their surgery at a specialised sarcoma hospitals. There is likely to be better control of the sarcoma at the original site, better recovery from the surgery and longer survival. There is also some evidence to support less chance of the sarcoma returning at the original site and less side effect when the radiotherapy is given at a specialised sarcoma hospital.

Ewing sarcoma in the pelvis

The guidelines also addressed a very specific question on the timing of surgery for patients with localised Ewing sarcoma in the pelvic bone. These patients are generally treated with chemotherapy first, followed by surgery and/or radiotherapy before further chemotherapy. Evidence showed that the delay of the surgery beyond 4 months from the start of chemotherapy appeared to have a negative impact on survival. The guidelines therefore recommend avoiding delay in the surgery (alone or with radiotherapy), and the treating multidisciplinary team should work closely during the initial chemotherapy period to coordinate the best time for surgery.