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.
软组织肉瘤是一种起源于中胚层的罕见肿瘤，可发生于肌肉、脂肪及神经等多种组织。该疾病包含超过100种亚型，各亚型具有不同的临床特征及治疗反应。近年来治疗进展已转向组织学特异性策略，强调整合病理学、免疫组化及分子特征以指导治疗。局限性软组织肉瘤主要采用手术治疗，常辅以新辅助或辅助放疗和/或化疗。然而约半数局限性病变患者会进展至晚期阶段，通常需采用全身性治疗方案，包括以多柔比星或表柔比星为代表的蒽环类化疗。尽管存在这些治疗手段，大多数晚期转移性软组织肉瘤亚型的生存率仍相对较低。虽然蒽环类化疗仍是治疗基石，但针对软组织肉瘤生物学特性的持续研究正深化我们对这类复杂肿瘤的认知与诊疗策略，治疗范畴已从化疗扩展至靶向治疗和免疫治疗，以期提高缓解率并改善生存结局。本综述聚焦于胃肠道间质瘤之外的软组织肉瘤，系统梳理当前全身治疗策略，重点阐述最新进展，并探讨肉瘤患者全身治疗的未来发展方向。
Systemic Treatment in Soft Tissue Sarcomas: Are We Making a Difference?
肿瘤(癌症)患者之家