Departments of Orthopedics, 71041National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Cancer Control. 2021 Jan-Dec;28:10732748211038424. doi: 10.1177/10732748211038424.
Soft tissue sarcomas are a form of rare and heterogeneous neoplasms with high recurrence rate and mortality. Over the past decades, less progress has been achieved. Surgical management with or without adjuvant/neoadjuvant radiotherapy is still the first-line treatment for localized soft tissue sarcomas, and chemotherapy is the additional option for those with high-risk. However, not all patients with advanced or metastatic soft tissue sarcomas benefit from conventional chemotherapy, targeted therapy takes the most relevant role in the management of those resistant to or failed to conventional chemotherapy. Heterogeneous soft tissue sarcomas vary from biological behavior, genetic mutations, and clinical presentation with a low incidence, indicating the future direction of histotype-based even molecule-based personalized therapy. Furthermore, increasing preclinical studies were carried out to investigate the pathogenesis and potential therapeutic targets of soft tissue sarcomas and increasing new drugs have been developed in recent years, which had started opening new doors for clinical treatment for patients with advanced/metastatic soft tissue sarcomas. Here we sought to summarize the concise characteristics and advance in the targeted therapy for the most common subtypes of soft tissue sarcomas.
软组织肉瘤是一种罕见且异质性的肿瘤，具有较高的复发率和死亡率。在过去的几十年中，进展甚微。手术治疗（包括辅助/新辅助放疗）仍然是局限性软组织肉瘤的一线治疗方法，而对于高危患者，化疗是额外的选择。然而，并非所有晚期或转移性软组织肉瘤患者都能从常规化疗中获益，靶向治疗在那些对常规化疗耐药或失败的患者的治疗中发挥着最重要的作用。不同的软组织肉瘤在生物学行为、遗传突变和临床表现上存在异质性，发病率较低，这表明基于组织类型甚至基于分子的个体化治疗将是未来的方向。此外，越来越多的临床前研究旨在探讨软组织肉瘤的发病机制和潜在治疗靶点，近年来也开发了越来越多的新药，为晚期/转移性软组织肉瘤患者的临床治疗开辟了新的途径。在这里，我们试图总结最常见的软组织肉瘤亚型的靶向治疗的简明特征和进展。
