Pathologic Angiogenesis of Malignant Vascular Sarcomas: Implications for Treatment

Khan, J. A., Maki, R. G., Ravi, V. (January 2018) Pathologic Angiogenesis of Malignant Vascular Sarcomas: Implications for Treatment. J Clin Oncol, 36 (2). pp. 194-201. ISSN 0732-183x

DOI: 10.1200/jco.2017.74.9812


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-beta 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.

Item Type: Paper
Subjects: diseases & disorders > cancer > angiogenesis
diseases & disorders > cancer > cancer types > angiosarcoma
diseases & disorders > cancer > cancer types > sarcoma
CSHL Authors:
Communities: CSHL Cancer Center Program > Signal Transduction
CSHL labs > Maki lab
CSHL Cancer Center Program > Cancer Genetics and Genomics Program
Depositing User: Matt Covey
Date: 10 January 2018
Date Deposited: 14 Dec 2017 16:01
Last Modified: 05 Nov 2020 21:07
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