Where the tumour is localised, wide excision (which implies that the surgeon cuts cleanly around the tumour, never through it, and with generous margins of safety) is recommended. Sometimes this requires amputation to achieve this goal but nowadays more often than not the requirement can be fulfilled by subablative surgery, perhaps supplemented by radiotherapy to the area post-operatively.
Where the sarcoma is localised to one muscle group (e.g. hamstrings of the thigh) such wide excision implies the resection of the whole muscle compartment (operation nicknamed, compartmentectomy), which can be a large and functionally disabling operation. Nevertheless, as an alternative to amputation, it is an advance over what was recommended twenty years ago, and with modern orthopaedic rehabilitation it is often less disabling than initially envisaged.
Where the operation is less than ablative, it is clear that the delivery of high radiotherapy to the region will further lessen the chances of relapse, and most patients will be recommended to receive a five to six week course of fractionated radiotherapy on modern equipment preceded by careful planning.
However, radiotherapy should not substitute a larger operation if the surgeon cannot achieve clear margins (i.e. the histologist is not able to identify any tumour at the edges of the operation specimen) as this does not achieve the good results that can be achieved by a good, clear surgical result before radiotherapy.
For many soft tissue sarcomas of the body, it is not possible to achieve even the modest margins achieved by a compartmentectomy in the limbs; here radiotherapy in the post-operative period is even more important. Sometimes, the radiotherapy precedes the surgery to try to effect shrinkage and facilitate curative resection.
The subject of adjuvant chemotherapy (i.e. the delivery of chemotherapy in the post-operative period in patients who have ostensibly localised disease but are at high risk of subsequent relapse; see breast cancer section) is highly controversial in this disease.
Some trials demonstrate that the delivery of the best agents known against this disease (e.g. doxorubicin/adriamycin and cyclophosphamide/iphosphamide) for a finite number of exposures (e.g. six) immediately after the operation can lead to a reduced risk of subsequent relapse.
However, the risk reduction is not as well validated as for example in node positive breast cancer (see breast cancer section) and many other trials only show a relapse free survival advantage (c.f. an overall survival advantage) or no advantage at all. Thus whilst there is now good evidence to support the routine delivery of adjuvant chemotherapy to children and young adults with resected rhabdomyosarcomas, this practice is not well validated and is not standard best care for many or most of adult soft tissue sarcomas encountered in the clinic.
Chemotherapy is so much better established in the childhood rhabdomyosarcoma practice that it is common for therapy to commence with chemotherapy here and surgery to come in later when the tumour has shrunk right down.
Where the patient presents with metastatic disease, the use of chemotherapy is obviously more in demand and there is a method of auditing the response by serial scans. The same drugs are used.
Occasionally, a single metastasis occurs (e.g. in the lungs) and surgery to this and the primary is considered, but this is very much a minority of cases and usually the patient who presents with metastatic disease is treated with chemotherapy to try to contain the disease for as long as possible.