Kaposiform Hemangioendotheliom  (KHE)

What is kaposiform hemangioendotheliom?

Kaposiform hemangioendotheliom (KHE) is a very rare type of non-cancerous (benign) tumor that grows in the blood vessels. It can appear anywhere on the body. KHE looks like a birthmark and usually grows just under the skin. But in some cases, it can grow more deeply in the chest, stomach or bones. The tumor often grows very quickly, but it does not spread to other areas of the body. KHE is most often diagnosed in babies under a year old.

Some children with KHE develop Kasabach-Merritt phenomenon. This condition can cause the number of platelets in the blood to drop, and affect the blood’s ability to clot. It is more likely to occur if a child has a very large tumor or if it is growing deeper in the body.

What are the signs and symptoms of KHE?

The most common symptom of KHE is a lesion on the skin that may look like a birthmark. The lesion may be:

• Purple or deep red in color
• Swollen and painful
• Shiny and firm in texture
• Warm to the touch

What causes KHE?

Experts do not know what causes KHE. It is not known to run in families.

How is KHE treated?

Treatment for KHE will depend on the size and location of your child’s tumor. Some small tumors may go away on their own with no treatment Your team at Arkansas Children’s is experienced in caring for children with KHE and will work with you to come up with the best treatment plan for your child.

Treatment options include:

• Watching the tumor to see if it needs treatment or if it will go away.
• Medications to slow the tumor’s growth or relieve symptoms.
• A procedure called embolization to cut off blood supply to the tumor.
• Surgery to remove the tumor. This may be the best option for small tumors or those that are growing very quickly.