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Kaposiform hemangioendothelioma (KHE) is an uncommon vascular tumor that can be present at birth or appear in early infancy. Compared with common hemangiomas, KHE occurs less frequently in the head and neck region; typical locations are the trunk, shoulder, thigh, and retroperitoneum. KHE is often associated with Kasabach-Merritt phenomenon, a bleeding disorde due to trapping of platelets by the tumor.
The involved skin is deep reddish-purple, tense, and shiny. Tiny purple or red spots and a bruise-like discoloration overlie and are adjacent to the tumor. This bruised appearance may falsely suggest a diagnosis of child abuse.
Retroperitoneal KHE is associated with a high mortality rate.
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