Abstract
Kaposiform hemangioendothelioma (KHE) is a vascular tumor with poor prognosis. We
present a child with progressive disability, extreme pain, and autonomic dysfunction
due to a retroperitoneal KHE where radiologic characteristics were essential for diagnosis
and monitoring of response to therapy. He received sirolimus, and the symptomatology
resolved completely. Repeat MRIs revealed fast marked decrease in vascularity of the
tumor, although the volume was not significantly affected. We suggest that the sirolimus-induced
tumor de-vascularization may explain the clinical and coagulopathy improvement.
Keywords
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Article info
Publication history
Published online: January 14, 2015
Accepted:
January 5,
2015
Received in revised form:
December 4,
2014
Received:
August 27,
2014
Identification
Copyright
© 2015 Elsevier Inc. Published by Elsevier Inc. All rights reserved.
