Revised 17 May 2010 

1) Curative setting

Chemotherapy may be combined with surgery or radiation therapy in the curative management of some patients with sarcoma.

• Routine post-operative adjuvant chemotherapy for the treatment of soft tissue sarcomas continues to be a controversial.
• However, adjuvant chemotherapy is a routine component in treatment of many patients with primary bone tumors and some of the soft tissue sarcomas, which have been mainly associated with the Pediatric or late adolescent age groups, such as Ewings Sarcoma also known as peripheral neuroectodermal tumor(PNET), rhabdomyosarcoma – all subtypes, and intra-abdominal small round blue cell tumor.
• Generally adjuvant chemotherapy programs are initiated before definitive surgery and/or radiation therapy and is known as neo-adjuvant or pre-operative chemotherapy.
• Local management with surgery or radiation is generally undertaken after several cycles of chemotherapy and then the chemotherapy is completed. Therefore, the sarcoma team medical oncologist must remain an active member of the multi-disciplinary team approaching the new patient to facilitate prompt completion of investigations, initiation of chemotherapy and coordination of the patient management plan.
• In specific clinical circumstances, some patients will be offered high dose chemotherapy with total body irradiation and stem cell rescue as the culmination of their treatment program.
• Gastrointestinal stromal tumours (GIST)

2) Palliative Setting

Chemotherapy can be helpful for palliation in selected patients with metastatic disease who have

• symptomatic unresectable metastases
• rapidly progressing metastases or
• unresectable primary tumours.

Standard drug therapy is single agent Adriamycin (SAAVA) which despite a response rate of 25 - 30 % affords equivalent prolongation of survival as compared to more modern single agent protocols or combinations. There is no curative drug therapy for metastatic inoperable soft tissue sarcomas. In some patients with peripheral neuroectodermal tumours and rhabdomyosarcoma, cure can be achieved with chemotherapy. Occasionally high does chemotherapy with stem cell support may be offered in those instances.

New drug development remains a very high priority for oncologists treating metastatic sarcomas. BCCA oncologists participate in studies testing new chemotherapy drugs combinations and/or new drugs for the treatment of patients. Some of these new drugs are modifications of older drugs known to have some activity; others are new types of drugs, such as signal transduction inhibitors. The latter are very attractive for patients who have types of tumors with readily identifiable genetic markers, e.g. c-kit in GIST. Unfortunately not every patent is eligible for every study but available studies will be discussed with the individual eligible patient.