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Chap 22 - HEMANGIOENDOTHELIOMAS, ANGIOSARCOMAS, AND KAPOSI'S SARCOMA

Published online by Cambridge University Press:  01 March 2011

By
Markku Miettinen
Edited by
Markku Miettinen
Markku Miettinen
Affiliation:
Armed Forces Institute of Pathology, Washington DC
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Summary

This chapter includes borderline to low-grade malignant vascular tumors (hemangioendotheliomas) and malignant vascular tumors (angiosarcoma and Kaposi's sarcoma).

Hemangioendothelioma is the designation for a group of borderline or outright malignant vascular tumors. They are capable of aggressive local growth, recurrence, or both, and some, especially epithelioid hemangioendothelioma, can also metastasize.

Angiosarcomas (AS) are malignant endothelial neoplasms. They are very rare compared with hemangiomas and comprise <1% of all vascular tumors and only 1% to 2% of all sarcomas. ASs are essentially composed of malignant endothelial cells representing endothelial cell neoplasms. They include rare low-grade variants but most are the high-grade variety. The features that aid in recognition of an angiosarcoma are disorganized and anastomosing vascular channels, permeative growth versus lobulation of the vascular units, and multilayered, atypical endothelia, often with solid areas, and a general lack of pericytes of the vessel wall. Lymphangiosarcomas are not recognized separately. ASs are frequently multifocal, making it sometimes difficult to determine the primary site. Because of this multifocal tendency, angiosarcomas are excluded from sarcoma staging systems.

Radiation and environmental carcinogens play a causal role, the latter especially in hepatic ASs. By their location, the endothelial cells are directly exposed to circulating carcinogens. The genetic mechanisms related to the pathogenesis of AS are much less understood than those for many other sarcomas, however.

Kaposi's sarcoma is etiologically related to human herpesvirus 8, which is one of the strongest connections between a virus and human mesenchymal neoplasia.

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Information
Modern Soft Tissue Pathology
Tumors and Non-Neoplastic Conditions
 , pp. 617 - 647
Publisher: Cambridge University Press
Print publication year: 2010

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To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

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Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

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