Kaposiform Hemangioendothelioma (KHE)

What is kaposiform hemangioendothelioma?

Kaposiform hemangioendothelioma (KHE) may look like a birthmark, but is actually a rare benign tumor caused by the abnormal growth of blood vessels. It can appear anywhere on the body, often on the head and neck but also on the stomach, back, arms and legs — even inside the chest, abdomen or bones.

Although KHE can grow, it doesn't spread to other locations in a child's body. A mild form of KHE, called tufted angioma, is less likely to cause complications or require treatment.

How we care for kaposiform hemangioendothelioma

Because kaposiform hemangioendotheliomas are so rare, very few doctors have experience diagnosing and treating them. The Vascular Anomalies Center (VAC) at Boston Children's Hospital has evaluated more children with KHE than any other hospital in the world. The VAC's 25 physicians — representing 16 medical and surgical specialties — draw on those experiences to redefine the best therapies and achieve better long-term outcomes for children with KHE.

Our areas of innovation for kaposiform hemangioendothelioma

Physicians and researchers at the VAC have generated an extensive database of cases of vascular anomalies, including more than 250 cases of KHE. We are actively evaluating the best therapies for KHE and studying long- term outcomes after KHE. As our database grows, it will help shine more light on this rare and often misunderstood condition.

In 2015, we opened a clinical trial comparing vincristine and sirolimus in patients with KHE complicated by KMP. This randomized, controlled, multicenter trial involving several institutions around the country will answer questions about which of these medications is a better treatment for KHE.