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. 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. KHE is often associated with Kasabach-Merritt phenomenon, a bleeding disorder. Retroperitoneal KHE is associated with a mortality rate that is greater than 50%.
Add to the discussion