Department of Surgery, Gastric and Mixed Tumor Service, Memorial Sloan Kettering Cancer Center, 1275 York Avenue, H1220, New York, NY, USA.
Division of Surgical Oncology, Department of Surgery, Sarcoma Disease Team, Winship Cancer Institute, Emory University Hospital Midtown, Emory University School of Medicine, 550 Peachtree St, NEMOT, 9th floor, Atlanta, GA 30322, USA. Electronic address: https://twitter.com/@kencardonaMD.
Surg Oncol Clin N Am. 2022 Jul;31(3):419-430. doi: 10.1016/j.soc.2022.03.006.
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.
未分化多形性肉瘤 (UPS) 和黏液纤维肉瘤 (MFS) 是基因组复杂的肿瘤，常见于老年患者的四肢或躯干。它们可能代表了一种疾病谱，通过在 MFS 中观察到的黏液样基质和曲线状血管与 UPS 相区别。保肢手术是标准的治疗方法，尽管 MFS 的浸润性需要比 UPS 更宽的切除边缘。UPS 相反与远端复发的高风险相关，通常促使建议进行辅助化疗。在这两种组织学中，蒽环类药物为基础的治疗或吉西他滨和顺铂用于治疗晚期疾病;免疫疗法可能对一部分患者有益。
