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Pediatr Cardiol. 2013 Jan;34(1):194-7. doi: 10.1007/s00246-012-0280-1. Epub 2012 Mar 16.

Hemangioendothelioma: a rare case of a primary intracardiac tumor.

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Children's National Medical Center, 111 Michigan Avenue, Washington, DC 20010, USA.


Kaposiform hemangioendothelioma (KH) is a rare tumor of vascular origin that commonly affects the cutaneous tissues of the extremities. It can, however, affect the abdomen, thorax, head, or neck. Compared with juvenile hemangiomas, which tend to regress, KH tumors are locally aggressive and usually persist. Associated morbidity and mortality rates range from 12 to 30 % and typically are related to either compressive effects on surrounding vital structures or effects of the Kasabach-Merritt phenomenon [10, 11, 13]. To our knowledge, this report is the first to describe KH presenting as a primary intracardiac tumor.

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