Department of Dermatology, Kyoto University Graduate School of Medicine, Kyoto, Japan.
Curr Opin Oncol. 2018 Mar;30(2):107-112. doi: 10.1097/CCO.0000000000000427.
The present review aims to provide readers with the latest updates on the biology and clinical management of cutaneous angiosarcoma (cAS).
The genomic alteration of cAS is heterogeneous. Mutations are enriched in the mitosis-activated kinase (MAPK) pathway. Functional analysis has identified molecules that may serve as potential markers and therapeutic targets of angiosarcoma. These molecules include survivin, HSP90, FOXM1, miR-497-5p, KCa3.1, and miR210.This body of knowledge has not yet transferred to clinical practice. The mainstay of treatment for cAS remains surgery followed by postoperative radiotherapy. The efficacy of paclitaxel as an adjuvant chemotherapy is suggested.For patients with advanced cAS, paclitaxel is the treatment of choice. There are also second-line treatment options that are supported by evidence of varying strength. A multikinase inhibitor, pazopanib, has been assessed in several studies, most of which support its efficacy for angiosarcoma. Bevacizumab monotherapy may be effective for angiosarcoma. The efficacy of eribulin mesylate and trabectedin for angiosarcoma is currently being assessed. Recent publications highlighted the role of the immune system in the biology of cAS.
Future research efforts should focus on the following aspects of cAS: drug development directed at recent molecular targets, clinical trials designed specifically for patients with cAS, and the role of immunotherapy for cAS.
本文旨在为读者提供皮肤血管肉瘤（cAS）生物学和临床管理的最新进展。
cAS 的基因组改变具有异质性。突变在有丝分裂激活激酶（MAPK）通路中富集。功能分析鉴定了可能作为血管肉瘤潜在标志物和治疗靶点的分子。这些分子包括存活素、热休克蛋白 90（HSP90）、叉头框蛋白 M1（FOXM1）、miR-497-5p、KCa3.1 和 miR210。这些知识尚未转化为临床实践。cAS 的主要治疗方法仍然是手术切除联合术后放疗。紫杉醇作为辅助化疗的疗效被认为是有效的。对于晚期 cAS 患者，紫杉醇是首选治疗方法。也有一些二线治疗选择，这些选择得到了不同强度证据的支持。多激酶抑制剂帕唑帕尼在几项研究中进行了评估，其中大多数研究支持其对血管肉瘤的疗效。贝伐单抗单药治疗可能对血管肉瘤有效。甲磺酸艾日布林和托泊替康治疗血管肉瘤的疗效正在评估中。最近的出版物强调了免疫系统在 cAS 生物学中的作用。
未来的研究工作应集中在以下几个方面：针对新的分子靶点的药物开发、专门针对 cAS 患者的临床试验、以及免疫疗法在 cAS 中的作用。
