Tan Grace Fangmin, Chan Jason Yongsheng
Division of Medical Oncology, National Cancer Centre Singapore, Singapore.
Oncology Academic Clinical Program, Duke-NUS Medical School, Singapore.
Oncotarget. 2021 Sep 14;12(19):1953-1955. doi: 10.18632/oncotarget.27996.
Angiosarcomas are a group of aggressive tumors of vascular origin. Although thought to be a rare cancer constituting just 1-2% of all soft tissue sarcomas, recent observations suggest that angiosarcomas are more common amongst Asian populations as compared to the West, suggesting the possibility of distinct genetic or environmental triggers influencing its pathogenesis. Advances in genomic sequencing efforts have led to the discovery of ultraviolet mutation signatures and high tumor mutation burden as common features of angiosarcoma of the head and neck. In addition, multi-omic analyses integrated with clinical data identified 3 subtypes characterized by distinctive etiological and biological phenotypes, with potential implications on precision therapy. The systemic and local immune milieu, as well as the presence of "giant" tumor cells, was also recently demonstrated to influence clinical behavior and patient outcomes, further highlighting complexities of this disease. Improvements in next generation "omic"-based technologies are expected to improve our understanding of angiosarcoma and guide the development of precision oncology in this rare cancer.
血管肉瘤是一组起源于血管的侵袭性肿瘤。尽管被认为是一种罕见的癌症，仅占所有软组织肉瘤的1%-2%，但最近的观察表明，与西方相比，血管肉瘤在亚洲人群中更为常见，这表明可能存在不同的遗传或环境触发因素影响其发病机制。基因组测序工作的进展导致发现紫外线突变特征和高肿瘤突变负荷是头颈部血管肉瘤的常见特征。此外，与临床数据相结合的多组学分析确定了3种具有独特病因和生物学表型的亚型，对精准治疗具有潜在意义。全身和局部免疫环境以及“巨大”肿瘤细胞的存在最近也被证明会影响临床行为和患者预后，进一步凸显了这种疾病的复杂性。预计下一代基于“组学”的技术的改进将提高我们对血管肉瘤的理解，并指导这种罕见癌症的精准肿瘤学发展。
