Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma

Surg Oncol Clin N Am. 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006.

Abstract

Undifferentiated pleomorphic sarcoma (UPS) and myxofibrosarcoma (MFS) are genomically complex tumors commonly diagnosed in the extremities or trunk of elderly patients. They likely represent a spectrum of disease differentiated by myxoid stroma and curvilinear vessels observed in MFS but not in UPS. Limb-sparing surgery is the standard of care although the infiltrative nature of MFS mandates wider resection margins than are necessary for UPS. UPS are conversely associated with high risks of distal recurrence, often prompting recommendations for adjuvant chemotherapy. In both histologies, anthracycline-based therapies or gemcitabine and docetaxel are used to manage advanced disease; immunotherapy may be of benefit in a subset of patients.

Keywords: Limb-sparing surgery; Malignant fibrous histiocytoma; Myxofibrosarcoma; Soft tissue sarcoma; Undifferentiated pleomorphic sarcoma; systemic therapy.

Publication types

  • Review
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adult
  • Aged
  • Extremities / pathology
  • Fibrosarcoma* / surgery
  • Histiocytoma, Malignant Fibrous* / pathology
  • Humans
  • Sarcoma* / drug therapy
  • Sarcoma* / pathology
  • Soft Tissue Neoplasms* / pathology