What is Kaposiform Hemangioendothelioma?

Kaposiform hemangioendothelioma (KHE) is a rare, sometimes fast-growing vascular tumor that typically occurs during infancy or early childhood. These tumors are often associated with an overlying vascular lesion (birthmark) on the skin. These tumors are usually benign (not cancerous). KHE is not inherited, and the cause is not known.

Signs & Symptoms

KHE lesions most frequently appear on the head, neck, stomach, back, arms or legs. They typically have the following characteristics:

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

Diagnosis

Kaposiform hemangioendothelioma is a complex condition and should be assessed and diagnosed by a physician who specializes in vascular anomalies. Diagnostic testing may include:

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

Treatment

Treatment for KHE varies depending on each child’s 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 some cases in which the tumor can be safely removed

The presence of Kaposiform hemangioendothelioma raises the risk of developing Kasabach-Merritt phenomenon, a rare condition that can be life-threatening.

Why Choose Us?

The vascular anomalies program at Comer Children's offers an integrated and comprehensive approach to the diagnosis, care and management of vascular malformations. Our multidisciplinary team of pediatric experts works together to educate families and to evaluate and treat children with all types of these vascular lesions.