Paudel Amrit, Chattopadhyay Priya, Rose Brandon, Watson Aleksandra, D'Amato Gina, Trent Jonathan, Bialick Steven, Jonczak Emily
Department of Medicine, Division of Medical Oncology, Sylvester Comprehensive Cancer Center, University of Miami, Miami, FL 33136, USA.
Department of Internal Medicine, Jackson Health System, University of Miami, Miami, FL 33136, USA.
Cancers (Basel). 2025 Mar 5;17(5):889. doi: 10.3390/cancers17050889.
Soft tissue sarcomas [STSs] are rare tumors of mesodermal origin that arise in diverse tissues such as muscles, fat, and nerves. There are over 100 subtypes of STS, each with distinct clinical behaviors and responses to treatment. Recent advances in treatment have moved towards histology-specific approaches, emphasizing the integration of pathological, immunohistochemical, and molecular features to guide treatment. Localized STS is primarily treated with surgery, often supplemented by neoadjuvant or adjuvant radiation and/or chemotherapy. However, about half of patients with localized disease will progress to an advanced stage, which is typically managed with systemic therapies including anthracycline-based chemotherapy such as doxorubicin or epirubicin. Despite these treatments, the survival rates for most subtypes of advanced metastatic STS remain relatively low. While anthracycline-based chemotherapy remains the mainstay of treatment, ongoing research into the biology of STSs is enhancing our understanding and approach to these complex tumors with an expansion beyond chemotherapy to include targeted therapy and immunotherapy to improve response rates and survival outcomes. This review focuses on STS other than gastrointestinal stromal tumors [GISTs], examines the current systemic treatment strategies, highlights recent advances, and explores future directions in the systemic therapy of sarcoma patients.
软组织肉瘤（STSs）是起源于中胚层的罕见肿瘤，可发生于多种组织，如肌肉、脂肪和神经。STSs有100多种亚型，每种亚型都有独特的临床行为和对治疗的反应。近年来治疗方法的进展朝着组织学特异性方法发展，强调整合病理、免疫组化和分子特征以指导治疗。局限性STSs主要通过手术治疗，通常辅以新辅助或辅助放疗和/或化疗。然而，约一半的局限性疾病患者会进展到晚期，晚期通常采用全身治疗，包括基于蒽环类药物的化疗，如多柔比星或表柔比星。尽管有这些治疗方法，但大多数晚期转移性STSs亚型的生存率仍然相对较低。虽然基于蒽环类药物的化疗仍然是主要的治疗方法，但对STSs生物学的持续研究正在加深我们对这些复杂肿瘤的理解和治疗方法，治疗范围已从化疗扩展到包括靶向治疗和免疫治疗，以提高缓解率和生存结果。本综述聚焦于非胃肠道间质瘤（GISTs）的STSs，研究当前的全身治疗策略，突出近期进展，并探索肉瘤患者全身治疗的未来方向。
