Angiosarcoma, epithelioid hemangioendothelioma, and Kaposi sarcoma are classified according to the line of differentiation that these neoplastic cells most closely resemble: the endothelial cell. Although these malignant vascular sarcomas demonstrate immunohistochemical and ultrastructural features typical of this lineage, they vary dramatically in presentation and behavior, reflecting oncologic mechanisms unique to each. Antineoplastic therapies offer significant benefit, but because of the rarity of these cancers, novel therapies are slow to develop, and treatment options for these cancers remain limited. Antiangiogenic approaches that have shown benefit in other malignancies have not fully realized their promise in vascular tumors, suggesting that these tumors do not depend entirely on either angiogenic growth factors or on neighboring endothelia that are affected by these agents. Nonetheless, translational studies have begun to unravel these distinct pathologies, identifying novel translocation products, targets of oncogenic virulence factors, and genomic mutations that hijack angiogenic signaling and drive malignant growth. Concurrently, an elaborate and highly regulated model of angiogenesis and lymphangiogenesis involving vascular endothelial growth factor-receptor tyrosine kinase and TGF-β and Notch pathways has emerged that informs treatment of these tumors as well as cancer in general. This review summarizes the literature on malignant vascular sarcomas in the context of current models of angiogenesis and, in light of recent clinical trial data, could help clinician-scientists generate novel therapeutic approaches.

中文翻译：

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恶性血管肉瘤的病理性血管生成：治疗意义

血管肉瘤、上皮样血管内皮瘤和卡波西肉瘤根据这些肿瘤细胞最相似的分化线进行分类：内皮细胞。尽管这些恶性血管肉瘤表现出该谱系典型的免疫组织化学和超微结构特征，但它们的表现和行为差异很大，反映了各自独特的肿瘤学机制。抗肿瘤疗法提供了显着的益处，但由于这些癌症的罕见性，新疗法的开发进展缓慢，并且这些癌症的治疗选择仍然有限。已在其他恶性肿瘤中显示出益处的抗血管生成方法尚未完全实现其在血管肿瘤中的前景，表明这些肿瘤不完全依赖于血管生成生长因子或受这些药物影响的邻近内皮细胞。尽管如此，转化研究已经开始解开这些不同的病理，确定新的易位产物、致癌毒力因子的靶点，以及劫持血管生成信号和驱动恶性生长的基因组突变。同时，出现了涉及血管内皮生长因子受体酪氨酸激酶和 TGF-β 和 Notch 通路的精细且高度调节的血管生成和淋巴管生成模型，可以为这些肿瘤以及一般癌症的治疗提供信息。本综述在当前血管生成模型的背景下总结了关于恶性血管肉瘤的文献，并根据最近的临床试验数据，