Abstract：
Angiosarcoma is a rare endothelial-derived malignancy that is extremely diverse in anatomy, aetiology, molecular and immune characteristics. While novel therapeutic approaches incorporating targeted agents and immunotherapy have yielded significant improvements in patient outcomes across several cancers, their impact on angiosarcoma remains modest. Contributed by its heterogeneous nature, there is currently a lack of novel drug targets in this disease entity and no reliable biomarkers that predict response to conventional treatment. This review aims to examine the molecular and immune landscape of angiosarcoma in association with its aetiology, anatomical sites, prognosis and therapeutic options. We summarise current efforts to characterise angiosarcoma subtypes based on molecular and immune profiling. Finally, we highlight promising technologies such as single-cell spatial \"omics\" that may further our understanding of angiosarcoma and propose strategies that can be similarly applied for the study of other rare cancers.
摘要：
血管肉瘤是一种罕见的内皮源性恶性肿瘤，在解剖学上极为多样化，病因学，分子和免疫特性。虽然结合靶向药物和免疫疗法的新型治疗方法在几种癌症的患者预后方面取得了显着改善，它们对血管肉瘤的影响仍然不大。由于其异质性，目前在该疾病实体中缺乏新的药物靶标,也没有可靠的生物标志物来预测对常规治疗的反应.这篇综述旨在研究血管肉瘤的分子和免疫景观及其病因。解剖部位,预后和治疗选择。我们总结了当前基于分子和免疫谱分析表征血管肉瘤亚型的努力。最后,我们重点介绍了诸如单细胞空间\"组学\"等有前景的技术,这些技术可能会进一步加深我们对血管肉瘤的理解,并提出可以类似地应用于其他罕见癌症研究的策略.
