Kaposiform Hemangioendothelioma (KHE) | Diagnosis & Treatment

How is kaposiform hemangioendothelioma diagnosed?

The first step in treating your child is forming an accurate and complete diagnosis. An experienced vascular anomalies specialist can help tell the difference between KHE and other vascular anomalies.

Most often, kaposiform hemangioendothelioma (KHE) is diagnosed through a complete medical history, a thorough physical exam and one or more of the following tests:

What are the treatments for kaposiform hemangioendothelioma?

Some mild KHE tumors go away without treatment. For those that do not, doctors have multiple treatment options available to them, depending on the size, location and behavior of the tumor. The goals of treatment are to relieve symptoms, shrink the tumor and reverse Kasabach-Merritt phenomenon, (KMP) if it is present.

Treatment options include:

  • Watchful waiting: For superficial tumors without low platelets, your child’s doctor may want to observe the tumor for a few months before determining the most appropriate treatment.
  • Oral or topical steroids: In mild cases, oral or topical steroids may help other treatments work better and improve superficial lesions. However, only about 12 percent of KHE tumors respond to steroids.
  • Drug therapy: Considered the gold standard treatment for most tumors, medications slow the growth of the tumor and improve symptoms.
  • Surgery: Your doctor will only recommend surgery if your child’s tumor is very small or extremely aggressive and able to be completely removed.
  • Embolization: This minimally invasive procedure blocks the main artery feeding a KHE tumor to cut off the blood supply and shrink it. We do not attempt embolization in cases where several arteries feed into the tumor, which is often the case with KHE.

Medications

Medications used to treat KHE include:

  • Sirolimus: Also known as rapamycin, this oral medication suppresses the immune system and slows the growth of abnormal lymphatic vessels that form the tumor. It has been found to shrink KHE tumors and improve symptoms, including pain.
  • Vincristine: This chemotherapy drug targets all dividing cells within the body and is therefore used to treat many cancers. While KHE is not a cancer, there are dividing cells in the tumor. Vincristine has been used successfully for decades to treat KHE.

What is the long-term outlook for a child with kaposiform hemangioendothelioma?

Many patients have an excellent long-term outlook through successful KHE treatment. In general, you child's prognosis depends on:

  • how quickly the diagnosis is made and appropriate medical therapy is started
  • the size, location and extent of disease
  • the tumor's initial response to therapy

When KHE is suspected, it’s important to see a specialist quickly to allow early discussions about treatment. Specialists can then attempt to shrink the tumor before it affects muscles or other tissues nearby. Early treatment could also help reduce long-term complications by preventing further growth.