Kaposiform Hemangioendothelioma (KHE)

Kaposiform hemangioendothelioma (KHE) is a rare and aggressive vascular tumor that is benign (noncancerous and does not spread to other locations in the body). It is an abnormal growth made up of blood vessels.

The outcome depends on:

  • Where on the body it is located
  • How large it is
  • How severely it causes a low blood platelet count (platelets, or thrombocytes, are blood cells that help blood to clot, which stops bleeding.)

Patients can be seen by Texas Children's experts in plastic surgery and vascular anomalies.

Causes and Risk Factors

The cause is not known.

Symptoms and Types

KHE appears as a single lesion at birth or during early infancy, usually on the trunk, arms or legs although the lesion can also appear on the head and the neck.

Symptoms include:

  • Deep reddish-purple color
  • Shiny, firm texture
  • Warm to the touch
  • Swollen and painful

Diagnosis and Tests

KHE is a complex condition. It should be assessed by a doctor who specializes in vascular tumors.

Testing may include:

  • Blood tests
  • Biopsy
  • Ultrasound
  • MRI or CT imaging

It is important to rule out thrombocytopenia, which is the medical term for a low blood platelet count.

Treatment and Care

Treatment varies depending on the symptoms and the extent of the disease.

Treatment may include:

  • Medications to slow the tumor's growth, or to shrink the tumor
  • Surgery may be appropriate in cases in which the tumor can be safely removed

Some large lesions cannot be completely removed. In these cases, several medical therapies may be used:

  • Systemic corticosteroids
  • Alpha interferon
  • Radiation therapy
  • Embolization

Living and Managing

It is essential to check the lesions often for a potentially fatal complication, Kasabach-Merritt syndrome.

This condition occurs when the tumor traps and destroys platelets in the blood, which leads to an increased risk of bleeding.

Approximately 80% of cases of Kasabach-Merritt syndrome occur within the first year of life.

Symptoms of Kasabach-Merritt syndrome may include:

  • Giant hemangioma, usually on the arm or leg,  often appearing as a large irregular bruise with shiny and dusky purple skin
  • Enlarged abdomen
  • Petechiae (pinpoint, round spots that appear because of bleeding under the skin), bruising and bleeding
  • Painful, tender lesions

Related Topics