血管肉瘤是一种在血管或淋巴管中发生的癌症，由于其罕见性和侵袭性特征而提出了重大的临床挑战。几十年来临床结果没有改善，突出表明需要创新的治疗策略来治疗该疾病。在遗传学上，血管肉瘤表现出高度异质性和复杂性，并具有许多复发性突变。然而，最近的研究发现了解剖学和分子亚组中的一些共同特征。为了识别潜在的治疗漏洞，必须了解血管肉瘤的突变情况并将其与现有的研究该疾病的模型相结合。在这篇综述中，我们将总结从报告的血管肉瘤分子和解剖亚型基因组改变中获得的见解，讨论几个潜在的可操作靶点，并重点介绍该领域可用的临床前疾病模型。© 2024 作者。约翰·威利出版的《分子肿瘤学》

Angiosarcoma is a cancer that develops in blood or lymphatic vessels that presents a significant clinical challenge due to its rarity and aggressive features. Clinical outcomes have not improved in decades, highlighting a need for innovative therapeutic strategies to treat the disease. Genetically, angiosarcomas exhibit high heterogeneity and complexity with many recurrent mutations. However, recent studies have identified some common features within anatomic and molecular subgroups. To identify potential therapeutic vulnerabilities, it is essential to understand and integrate the mutational landscape of angiosarcoma with the models that exist to study the disease. In this review, we will summarize the insights gained from reported genomic alterations in molecular and anatomic subtypes of angiosarcoma, discuss several potential actionable targets, and highlight the preclinical disease models available in the field.© 2024 The Author(s). Molecular Oncology published by John Wiley & Sons Ltd on behalf of Federation of European Biochemical Societies.