Miwa Shinji, Yamamoto Norio, Hayashi Katsuhiro, Taniguchi Yuta, Yonezawa Hirotaka, Morinaga Sei, Demura Satoru
Department of Orthopaedic Surgery, Graduate School of Medical Sciences, Kanazawa University, 13-1 Takara-Machi, Kanazawa, 920-8640, Japan.
Int J Clin Oncol. 2025 May 14. doi: 10.1007/s10147-025-02712-6.
Undifferentiated pleomorphic sarcoma (UPS)/malignant fibrous histiocytoma (MFH) is the second most common soft-tissue sarcoma. The standard treatment options for UPS/MFH include tumor excision with appropriate surgical margins, radiation therapy, and chemotherapy. Preferable clinical outcomes can be expected in patients with resectable disease, whereas the clinical outcomes in patients with metastatic disease are unsatisfactory despite multidisciplinary treatment. Although patients with metastatic diseases require chemotherapy, the response rate to conventional chemotherapy has been reported to be only 27-33% in previous reports. Systemic treatment is required to eliminate metastatic disease and improve clinical outcomes in patients with UPS/MFH. Recent clinical studies have investigated the optimal period of conventional chemotherapy and the efficacy of various combinations of anticancer agents. Furthermore, molecular targeted drugs and immune checkpoint inhibitors have shown superior outcomes compared to standard treatments for various types of malignancies. Therefore, these anticancer agents are considered as new treatment options for patients with UPS/MFH. Recent clinical trials have demonstrated the safety and efficacy of these agents in patients with soft-tissue sarcomas, including UPS/MFH. In particular, a high response rate to immune checkpoint inhibitors combined with doxorubicin has been reported in recent clinical trials; however, combination therapy needs to be assessed in a large number of patients with UPS/MFH. In this review article, recent clinical studies on the systemic treatment of UPS/MFH are discussed.
未分化多形性肉瘤（UPS）/恶性纤维组织细胞瘤（MFH）是第二常见的软组织肉瘤。UPS/MFH的标准治疗方案包括进行具有适当手术切缘的肿瘤切除、放射治疗和化疗。可切除疾病患者有望获得较好的临床结局，而转移性疾病患者尽管接受了多学科治疗，其临床结局仍不尽人意。虽然转移性疾病患者需要化疗，但既往报道显示对传统化疗的缓解率仅为27%-33%。需要进行全身治疗以消除转移性疾病并改善UPS/MFH患者的临床结局。近期的临床研究调查了传统化疗的最佳疗程以及各种抗癌药物联合使用的疗效。此外，与各种类型恶性肿瘤的标准治疗相比，分子靶向药物和免疫检查点抑制剂已显示出更优的疗效。因此，这些抗癌药物被视为UPS/MFH患者的新治疗选择。近期的临床试验已证明这些药物在包括UPS/MFH在内的软组织肉瘤患者中的安全性和有效性。特别是，近期临床试验报道了免疫检查点抑制剂联合阿霉素具有较高的缓解率；然而，联合治疗需要在大量UPS/MFH患者中进行评估。在这篇综述文章中，将讨论近期关于UPS/MFH全身治疗的临床研究。
