Best results require complete eradication of the primary tumour by radical surgery combined with chemotherapy.
Amputation should ideally extend through or above the joint proximal to the tumour, including all affected muscle but may be unacceptable. More restricted amputation or a wide excision with limb sparing, may be performed only if the patient is aware of the increased likelihood of recurrence.
Chemotherapy is started pre-operatively, usually with methotrexate. Its effectiveness is then reassessed following surgery and another agent may be substituted.
Radiotherapy is restricted to tumours in inoperable sites - for example, the pelvis or jaw - and to those for whom amputation is unacceptable.
Lung secondaries are resected wherever possible.
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