Background/Objectives: Cutaneous angiosarcoma (CAS) is a rare and aggressive endothelial malignancy with a high rate of local recurrence and distant metastasis. In advanced cases, where surgical resection is not feasible, systemic therapy remains the cornerstone of treatment. This review aims to summarize the current landscape of systemic therapies for unresectable or metastatic CAS and discuss emerging strategies, particularly focusing on immune checkpoint inhibitors (ICIs).Methods: A comprehensive review of the literature was conducted, including clinical trials, retrospective studies, and case series focusing on systemic treatments for advanced CAS. Therapeutic approaches covered include cytotoxic chemotherapy, molecular targeted therapies, and ICIs, as well as combination strategies. Special attention was given to biomarker studies and ongoing clinical trials.Results: Taxane-based chemotherapy, particularly paclitaxel, has demonstrated clinical activity and remains a standard option. Molecular targeted agents such as pazopanib have yielded modest efficacy. Recent trials of ICIs, including the SWOG S1609 DART and AngioCheck studies, have shown encouraging results in select subgroups, especially tumors from sun-exposed regions associated with high tumor mutational burden (TMB). Although AngioCheck did not meet its predefined response criteria, a subset of patients achieved disease control. Biomarkers such as TMB, PD-L1 expression, and tumor-infiltrating lymphocytes are under investigation to guide patient selection. Combination therapies with ICIs and tyrosine kinase inhibitors (TKIs) are being actively explored.Conclusions: While systemic therapies for CAS remain limited in efficacy, ICIs—particularly in combination with TKIs—represent a promising avenue. Future trials should emphasize biomarker-driven, CAS-specific strategies to improve clinical outcomes in this challenging malignancy.
**背景/目的：** 皮肤血管肉瘤是一种罕见且侵袭性强的内皮细胞恶性肿瘤，局部复发和远处转移率高。对于手术切除不可行的晚期病例，全身性治疗仍是治疗的基石。本综述旨在总结目前针对不可切除或转移性皮肤血管肉瘤的全身性治疗现状，并讨论新兴策略，尤其聚焦于免疫检查点抑制剂。 **方法：** 对文献进行了全面回顾，包括关注晚期皮肤血管肉瘤全身性治疗的临床试验、回顾性研究和病例系列。涵盖的治疗方法包括细胞毒性化疗、分子靶向治疗、免疫检查点抑制剂以及联合策略。特别关注了生物标志物研究和正在进行的临床试验。 **结果：** 以紫杉烷类为基础的化疗，特别是紫杉醇，已显示出临床活性，并仍是标准选择。帕唑帕尼等分子靶向药物疗效有限。最近的免疫检查点抑制剂试验，包括SWOG S1609 DART和AngioCheck研究，在特定亚组中显示出令人鼓舞的结果，尤其是与高肿瘤突变负荷相关的日光暴露区域来源的肿瘤。尽管AngioCheck研究未达到预设的应答标准，但一部分患者实现了疾病控制。目前正在研究肿瘤突变负荷、PD-L1表达和肿瘤浸润淋巴细胞等生物标志物，以指导患者选择。免疫检查点抑制剂与酪氨酸激酶抑制剂的联合疗法正在积极探索中。 **结论：** 尽管皮肤血管肉瘤的全身性治疗效果仍然有限，但免疫检查点抑制剂——特别是与酪氨酸激酶抑制剂联合——代表了一条有前景的途径。未来的试验应强调基于生物标志物的、针对皮肤血管肉瘤的特异性策略，以改善这一具有挑战性的恶性肿瘤的临床结局。
肿瘤(癌症)患者之家