Locally Aggressive or borderline vascular tumors (formerly rare benign tumors)

Kaposiform hemangiomaendothelioma (KHE)


KHE is an uncommon vascular tumor that most often presents at birth or in the first weeks of life.  This type of vascular tumor is often found to occur in the trunk, shoulder, thigh and abdominal cavity.


KHE tumors appear as deep reddish to purple lesions.  The skin is often tense and shiny.  Small purple or red spots may appear adjacent to the lesion.  Some of these spots may lie within the boundaries of the lesion.  The lesion often has a “bruised” look to it.


KHE is associated with a bleeding disorder known as Kasabach-Merritt phenomenon.  This disorder results from platelets being trapped within the tumor.  (thrombocytopenia).  Other important clotting factors are also depleted in Kasabach-Merritt phenomenon.  Blood tests showing platelets and fibrinogen levels can show if thrombocytopenia is occurring.  Kasabach-Merritt occurs in KHE and Tufted Angioma but never occurs in infantile hemangioma.


KHE tumors occurring in the abdominal cavity (retroperitoneal) are associated with a high mortality rate.


KHE lesions sometimes respond to treatment with corticosteroids.  Often the drug vincristine is needed to control the tumor growth and manage the bleeding disorder.


Tufted Angioma, (TA)


Another rare vascular tumor that occurs in infancy and early childhood is Tufted Angioma.  TA tumors appear as a small red or purple patches on the skin.  They most often occur in the neck and upper trunk regions.  TA grows slowly but may cover large areas of the neck and trunk.


TA is associated with Kasabach-Merritt phenomenon.  Symptoms are the same as in KHE and are treated with corticosteroids or vincristine.


NOVA provides this information as a resource.  It is not intended to engage in the practice of medicine or to replace the physician.  NOVA does not claim to have medical knowledge.  NOVA does not endorse any particular physician, treating facility or treatment protocol.  In all cases NOVA, the NOVA Board of Directors and associates recommends that you seek the opinion of a physician experienced in the diagnosis and treatment of hemangioma and vascular malformations.


Revised 04-5-2014


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