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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. 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, drug therapy 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 registries.

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 a diagnosis or suspected diagnosis of sarcoma. The scientific evidence suggests that outcomes are generally better when patients received their surgery at a specialised sarcoma hospital. 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.

The following information is for specific types of sarcoma

  1. Retroperitoneal Sarcoma

Retroperitoneal sarcoma is a sarcoma that occurs in the abdomen behind the peritoneum (the membrane lining the abdomen cavity and covering the abdominal organs). The two main subtypes of retroperitoneal sarcoma are liposarcoma (sarcoma that resembles fatty tissue) and leiomyosarcoma (sarcoma that resembles smooth muscle).  Patients with this diagnosis should be treated by a dedicated team of doctors from different specialties (multidisciplinary team, MDT) with expertise in sarcoma management. 

Obtaining a tissue sample (biopsy) to determine the exact type of sarcoma is recommended to plan treatment. The recommended biopsy method is coaxial biopsy (a system with an outer guiding needle and an inner biopsy needle). Using this technique to obtain tissue sample does not increase the chance of the sarcoma returning.

For patients with localised disease, the main curative treatment is complete surgical removal of the disease. Multivisceral resection (surgery to remove the organs adjacent to the sarcoma mass) may help to get a better margin which in turn may help to reduce the chance of the sarcoma returning inside the abdomen. This type of surgery is relatively safe.

For patients with first diagnosis of localised retroperitoneal sarcoma, the use of radiotherapy before surgery may be considered for certain subtypes of sarcoma such as low grade or intermediate grade liposarcoma (sarcoma that resembles fatty tissue) to reduce the chance of the sarcoma returning inside the abdomen. The use of radiation therapy in addition to surgery is not recommended for other types of sarcoma. The use of chemotherapy in addition to surgery is also not recommended except in a clinical trial setting.

  1. 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.

  1. High dose chemotherapy for Ewing Sarcoma and Rhabdomyosarcoma

This series also addressed a very specific question on the role of high dose chemotherapy with autologous stem cell transplant. This is a treatment protocol combining higher doses of chemotherapy with a bone marrow transplant. The high dose chemotherapy destroys the cancer cells in the body, as well as the normal cells in the bone marrow. The transplant (infusion of healthy bone marrow stem cells) will help the body to start producing healthy bone marrow cell again.

High dose chemotherapy in Ewing sarcoma

Ewing sarcoma is a type of primary bone cancer. It is most common in adolescents and young adults. The treatment often involves a combination of chemotherapy, surgery and/or radiotherapy. For patients where the Ewing sarcoma has returned after previous treatment, there may be a role for high dose chemotherapy especially if there has been a good response to second line chemotherapy prior to the high dose chemotherapy.

High dose chemotherapy in Rhabdomyosarcoma

Rhabdomyosarcoma is a sarcoma that usually begins in muscles but it may begin in many places in the body. It is the most common type of soft tissue sarcoma in children. These patients are generally treated with chemotherapy first, followed by surgery and/or radiotherapy before further chemotherapy. High dose chemotherapy has no proven benefit for patients with any stage of rhabdomyosarcoma.