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Treatment: Surgery is the mainstay of treatment for undifferentiated pleomorphic sarcomas (UPS). Surgical options fall into two categories: amputation and limb-sparing surgery. Historically, most UPS have been treated with amputation (as shown in the left panel). However, several studies have recently shown that amputation offers no survival advantage over limb-sparing surgery. Currently, the vast majority of UPS are treated by wide local excision (right panel).

Limb-sparing procedures are performed only if the surgeon is confident that the tumor can be removed completely and that the remaining limb will be functional. Reconstructive procedure on the bone or joint may be necessary after surgery. Post-operative external beam radiation therapy has been used to reduce the risk of local recurrences.

The role of chemotherapy is not entirely clear and is currently offered only to those with recurrent or metastatic disease. Recent studies have shown promising results with immune checkpoint inhibitors before surgery in patients with resectable UPS.

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