Some of the information within this section is taken from the ESMO clinical guidelines and its use is subject to permission from ESMO.
Surgical excision of the primary tumour is the treatment of choice. It should be the first treatment option when conservative resection is both feasible and oncologically effective: en bloc, macro and microscopically complete surgical excision of the gross tumour encompassing the biopsy scar (R0). The persistence of microscopic residual disease (R1 resection) is associated with a higher risk of relapse, and secondary cytoreduction should be considered when technically feasible.
Patients with macroscopically incomplete surgery have an extremely high risk of relapse and secondary surgical excision of residual disease is mandatory. For sarcomas of the extremities, surgery may require amputation, complete resection of the tumour bed, or wide local excision with negative tissue margins, depending on the cases.
Radiation therapy as an adjuvant to surgery is an option in selected cases of deep lesions £5 cm or low-grade tumors. Compartmental resection of an intracompartmental tumor, if performed, does not require adjuvant radiation therapy.