A kaposiform hemangioendothelioma is an uncommon vascular tumor that typically presents very early in life, although it may present months to years later. It is a locally aggressive tumor that often involves the underlying tissue.
Kaposiform hemangioendothelioma and tufted angioma are sometimes associated with the Kasabach-Merritt phenomenon, which is a clinical syndrome comprised of coagulopathy, thrombocytopenia, and microangiopathic hemolytic anemia due to a rapidly enlarging vascular tumor.
The most common tumors to cause this syndrome are kaposiform hemangioendothelioma and tufted angioma. Clinical findings associated with thrombocytopenia can be found, including bruising and bleeding, both internally and externally. This phenomenon is an emergency, as it may be life-threatening.
These tumors are difficult to treat and a multispecialty approach, such as the one offered by our Vascular Lesion and Birthmark Center, is necessary.