Chen Tom Wei-Wu, Burns Jessica, Jones Robin L, Huang Paul H
Department of Oncology, National Taiwan University Hospital and Graduate Institute of Oncology, National Taiwan University College of Medicine, Taipei 100, Taiwan.
Division of Molecular Pathology, The Institute of Cancer Research, London SW3 6JB, UK.
Cancers (Basel). 2020 Nov 10;12(11):3321. doi: 10.3390/cancers12113321.
Angiosarcomas comprise less than 3% of all soft tissue sarcomas but have a poor prognosis. Most angiosarcomas occur without obvious risk factors but secondary angiosarcoma could arise after radiotherapy or chronic lymphedema. Surgery remains the standard treatment for localized angiosarcoma but neoadjuvant systemic treatment may improve the curability. For advanced angiosarcoma, anthracyclines and taxanes are the main chemotherapy options. Anti-angiogenic agents have a substantial role but the failure of a randomized phase 3 trial of pazopanib with or without an anti-endoglin antibody brings a challenge to future trials in angiosarcomas. Immune checkpoint inhibitors as single agents or in combination with oncolytic virus may play an important role but the optimal duration remains to be investigated. We also report the current understanding of the molecular pathways involved in angiosarcoma pathogenesis including MYC amplification, activation of angiogenic pathways and different molecular alterations that are associated with angiosarcomas of different aetiology. The success of the patient-partnered Angiosarcoma Project (ASCProject) has provided not only detailed insights into the molecular features of angiosarcomas of different origins but also offers a template for future fruitful collaborations between patients, physicians, and researchers. Lastly, we provide our perspective of future developments in optimizing the clinical management of angiosarcomas.
血管肉瘤占所有软组织肉瘤的比例不到3%，但预后较差。大多数血管肉瘤的发生没有明显的危险因素，但继发性血管肉瘤可能在放疗或慢性淋巴水肿后出现。手术仍然是局限性血管肉瘤的标准治疗方法，但新辅助全身治疗可能会提高治愈率。对于晚期血管肉瘤，蒽环类药物和紫杉烷类药物是主要的化疗选择。抗血管生成药物发挥着重要作用，但帕唑帕尼联合或不联合抗内皮糖蛋白抗体的3期随机试验失败给未来血管肉瘤试验带来了挑战。免疫检查点抑制剂作为单一药物或与溶瘤病毒联合使用可能发挥重要作用，但最佳持续时间仍有待研究。我们还报告了目前对血管肉瘤发病机制中涉及的分子途径的理解，包括MYC扩增、血管生成途径的激活以及与不同病因血管肉瘤相关的不同分子改变。患者参与的血管肉瘤项目（ASCProject）的成功不仅提供了对不同起源血管肉瘤分子特征的详细见解，也为患者、医生和研究人员未来富有成效的合作提供了一个模板。最后，我们阐述了在优化血管肉瘤临床管理方面未来发展的观点。
